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Objectives: We aimed to outline the demographic data, clinical spectrum, and treatment approach of sarcoidosis in a large group of patients and sought to figure out the variations of early-onset (EOS) and late-onset paediatric sarcoidosis (LOS). Methods: The study followed a retrospective-descriptive design, with the analysis of medical records of cases diagnosed as paediatric sarcoidosis. Results: Fifty-two patients were included in the study. The median age at disease onset and follow-up duration were 83 (28.2–119) and 24 (6–48) months, respectively. Ten (19.2%) cases had EOS (before 5th birthday) and 42 (80.7%) cases had LOS. The most common clinical findings at the time of the disease onset were ocular symptoms (40.4%) followed by joint manifestation (25%), dermatological symptoms (13.5%), and features related to multi-organ involvement (11.5%). Anterior uveitis was the most common (55%) one among ocular manifestations. Patients with EOS displayed joint, eye, and dermatological findings more commonly than patients with LOS. The recurrence rate of disease in patients with EOS (5.7%) and LOS (21.1%) were not statistically different (P = .7). Conclusions: Patients with EOS and LOS may present with variable clinical features and studies addressing paediatric sarcoidosis cases in collaboration between disciplines will enhance the awareness of this rare disease among physicians and assist early diagnosis with lesser complications. [ABSTRACT FROM AUTHOR]

Takayasu's arteritis (TA) is a rare chronic granulomatous vasculitis characterized by large-vessel involvement. The aorta and its main branches are most commonly involved. Although pulmonary artery involvement is common, hemoptysis or respiratory findings are rarely seen. Herein, we present a case of TA who developed anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis with diffuse alveolar hemorrhage after coronavirus disease 2019 (COVID-19) infection. A 17-year-old female patient with the diagnosis of TA presented with cough, bloody vomiting, and diarrhea. In follow-up, she developed tachypnea and dyspnea and was transferred to the pediatric intensive care unit. The findings on the chest computed tomography were compatible with acute COVID-19 infection, but the SARS-CoV2 reverse transcription-polymerase chain reaction test was negative, but SARS-CoV2 immunoglobulin (Ig) G and IgM antibody tests were positive. The patient was not vaccinated against COVID-19. The bronchoscopy showed bronchial mucosal fragility, bleeding foci, and mucosal bleeding. The broncoalveolar lavage hemosiderin-laden macrophages were seen in the histopathologic examination. The indirect immunofluorescence assay-ANCA test became 3 (+) with myeloperoxidase (MPO)-ANCA of 125 RU/ml (normal: <20). Cyclophosphamide and pulse steroid treatment were started. After immunosuppressive therapy, the patient condition improved and did not have hemoptysis again. The successful response was obtained by applying balloon angioplasty to the patient with bilateral renal artery stenosis. Types of post-COVID vasculitis include thromboembolic events, cutaneous vasculitis, Kawasaki-like vasculitis, myopericarditis, and ANCAassociated vasculitis. It is thought that COVID-19 may impair immune tolerance and trigger autoimmunity with cross-reaction. To the best of our knowledge, the third pediatric case was reported with MPO-ANCA-positive COVID-associated ANCA vasculitis. [ABSTRACT FROM AUTHOR]

The article discusses a pediatric case of Immunoglobulin G4-related disease (IgG4-RD) with rare central nervous system involvement. Topics include the clinical presentation of IgG4-RD in a 17-year-old girl, the diagnostic challenges encountered, and the treatment approach using corticosteroids and mycophenolate mofetil.

OBJECTIVE: The objectives of this study were to determine the musculoskeletal (MSK) conditions associated with pediatric psoriasis (Pso) and to evaluate the thickness of Achilles tendon of children with Pso and healthy controls (HCs). METHODS: Pso patients who were followed-up in dermatology outpatient clinic were referred to a pediatric rheumatology center. All patients and healthy peers were evaluated with standardized forms. Both patients and controls underwent ultrasonographic evaluation for Achilles tendon thickness. RESULTS: A total of 55 pediatric Pso and 46 healthy children were included in the study. Of patients with Pso 56.4% had arthralgia, 25.5% had lower back pain, 18.2% had heel pain, 12.7% had hip pain, and 10.9% described morning stiffness. Arthritis was detected in 7.3%, sacroiliac tenderness in 12.7%, and enthesitis in 9.1% of the patients. Arthralgia, lower back pain, and heel pain were significantly frequent in Pso group than healthy children median left and right Achilles tendon thicknesses of Pso patients who were significantly greater than that of HCs prevalence of psoriatic arthritis (PsA) among Pso patients was 7.3%. CONCLUSION: Evaluation of a child with Pso regularly for the MSK complaints is critical for the early recognition of PsA. Ultrasonography is a useful technique for screening Pso patients for early detection of enthesopaty. [ABSTRACT FROM AUTHOR]

Objectives: To determine the rate of achieving The Lupus Low Disease Activity State (LLDAS) in children with systemic lupus erythematosus (SLE) for tracing pertinent treatment modalities. Methods: A total of 122 juvenile-onset SLE (jSLE) patients from six pediatric rheumatology centers in Turkey were enrolled in the study. LLDAS-50 was defined as encountering LLDAS for at least 50% of the observation time. According to the achievement of LLDAS-50, clinical features, immunological profiles, and treatments of patients with jSLE have been revealed. Results: LLDAS of any duration was achieved by 82% of the cohort. Although only 10.8% of the patients achieved remission, 68.9% reached LLDAS-50. A significant difference was found between patients who reached LLDAS-50 and those who did not, in terms of the time to reach low-dose corticosteroid treatment (p = 0.002), the presence of subacute cutaneous findings (p = 0.007), and the presence of proteinuria (p = 0.002). Both of the groups were under similar treatment approaches. However, the number of patients being treated with corticosteroids at the last visit was found to be significantly higher in patients who achieved LLDAS-50 (p <0.001). Conclusion: Targeting LLDAS in jSLE, even with long-term, low-dose corticosteroid use, seems to be an achievable goal in clinical practice. [ABSTRACT FROM AUTHOR]

OBJECTIVE: This retrospective observational study aims to demonstrate initial signs and symptoms of Henoch-Schönlein purpura (HSP), search for risk factors for gastrointestinal and renal involvement and give short term follow-up results. METHODS: The files of newly diagnosed HSP patients from two pediatric rheumatology centers in the southeastern part of the country were retrospectively analyzed in this study. Demographic, clinical features and laboratory results were recorded from the files. RESULTS: The cohort consisted of 323 children (males: 53.6%, females: 46.4%). Median age at the time of diagnosis was 7.5 (1.8-17.3) years and the median duration of follow-up was six (3-22) months. The rash was present in all cases but was not the first symptom in 22.9% of the cases. Arthritis and abdominal pain before the development of rash were the initial symptoms in 11.8% and 11.1% of the cases, respectively. Other manifestations were subcutaneous edema (63.2%), arthralgia (57.6%), arthritis (27.6%), myalgia (17.6%), lethargy (10.2%), orchitis (7.5%) and fever (5.3%). Gastrointestinal involvement was seen in 53.3% and renal involvement in 23.5% of the cases. None of the patients developed renal impairment during the follow-up. Older age at diagnosis, necrotic rash, subcutaneous edema, abdominal pain, lethargy, myalgia, arthralgia and arthritis were significantly higher in patients with renal involvement, but none of the demographic, clinical and laboratory features was an independent risk factor for renal or gastrointestinal involvement. CONCLUSION: Abdominal pain, arthritis may be the first manifestation of HSP. Having constitutional symptoms, such as fever, myalgia and lethargy at the time of diagnosis, may be warning signs of a more aggressive course with gastrointestinal and renal involvement. [ABSTRACT FROM AUTHOR]

OBJECTIVE: Juvenile idiopathic arthritis (JIA) is the most common cause of chronic arthritis in children. Biologics have changed the faith of children with rheumatic diseases. The main objective of this study was to demonstrate the rate of usage, efficacy and safety of biologics in JIA subtypes. METHODS: This retrospective observational cohort study was conducted between May 2010 and September 2017. All children with the diagnosis of JIA and children under a biological agent treatment were recorded into the local registry system. Age, gender, JIA subtype, medications used, the clinical status of the patient, tuberculosis screening results, and side effects observed under biologics were retrieved from the registry. RESULTS: There were 405 patients with the diagnosis of JIA in the cohort. Biologics were used in 123 (30.3%) JIA patients. Subtype frequencies of JIA patients were as follows: persistent oligoarticular JIA (33.6%), enthesitis-related arthritis (29.2%), systemic JIA (13%), rheumatoid factor (RF)-negative polyarticular JIA (13%), extended oligoarticular JIA (4.2%), RF-positive polyarticular JIA (3.4%), psoriatic arthritis (1.8%) and unclassified arthritis (1.8%). The rate of biologic use was high in extended oligoarticular JIA (64.7% of the cases), RF-positive polyarticular JIA (57.1%), psoriatic arthritis (57.1%), RFnegative polyarticular JIA (41.5%), and in systemic JIA (39.6%). Enthesitis-related arthritis (27.1%), persistent oligoarticular JIA (17.6%) and unclassified arthritis (16.6%) patients were the cases that needed a biologic agent in the last order. At the last control, 78.9% of the cases were in remission, while 21.1% of them were active despite biologic treatment. Isoniazid prophylaxis was used in 30.8% of the patients. None of the patients developed active tuberculosis infection under prophylaxis. Adverse events were observed in 18.6% of patients under biologics as recurrent uncomplicated upper respiratory tract infections being the most common. CONCLUSION: Biologics are safe and effective treatment options in children with JIA. Most of the JIA patients with polyarticular involvement require biologics earlier in the disease course. The risk of tuberculosis infection seems not to be increased after appropriate screening and prophylaxis. [ABSTRACT FROM AUTHOR]

Aim: To evaluate the macula, optic nerve and choroid layer by using Spectral Domain (SD)-OCT in children with joint hypermobility (JH). Material and Methods: In this cross-sectional case control study, 25 eyes of 25 children with JH diagnosed by rheumatological examination and 37 eyes of 37 healthy children were evaluated. Images were taken with SD -OCT. Two groups were compared in terms of mean central macular thickness, macular volume, ganglion cell complex (GCC) thickness, retina nerve fiber layer (RNFL) thickness and choroidal thickness (CT). CT was measured at the fovea; other CT measurements were taken 1,000 µm away from the fovea at the nasal and temporal regions and 1000 µm away from the optic disc at the nasal and temporal regions. Results: Mean subfoveal CT was 293.85±40.74 µm in JH group and 301.66±49.47 µm in the control group (p=0.548). There was no significant difference regarding to the mean macular and peripapillary CT values of all regions (P >0 .05 for all points). Moreover, the mean thicknesses of the RNFL, macula, and GCC did not differ between the two groups. Conclusion: This study showed that CT of children with JH is similar to those of healthy controls. In addition, there was no difference in macular thickness, RNFL and GCC between the two groups. These findings suggest that posterior segment structures are not affected in individuals with JH. [ABSTRACT FROM AUTHOR]

Familial Mediterranean fever (FMF) is the most common type of monogenic periodic fever syndromes and characterized by recurrent self-limited attacks of fever and polyserositis. Musculoskeletal signs and symptoms are not uncommon and manifested as arthritis and myalgia. Myalgia may be spontaneous or exercise-induced that mostly affects lower limbs and spontaneously resolves in 2-3 days. Protracted febrile myalgia syndrome (PFMS) is another form of rare and severe muscle involvement in FMF. PFMS affects all muscle groups and lasts for several weeks. Herein we present a pediatric case of PFMS that presented as the first manifestation of FMF, not responded to prednisolone at all but showed dramatic improvement with anakinra. Our case has a few distinctive points. She did not have a diagnosis of FMF and also she did not have any previous complaints compatible with FMF. Thus, PFMS was the first sign of FMF in this patient. Most of the cases of PFMS show dramatic response to corticosteroids, but our case did not respond at all to high-dose corticosteroids and anakinra resulted in rapid resolution of the symptoms. Protracted febrile myalgia syndrome may be the first manifestation of FMF. It should be suspected in cases with prolonged and unexplained fever, severe myalgia, and high acute phase reactants. [ABSTRACT FROM AUTHOR]

Alnaç: Eritema ab iğne tekrarlayan ısı maruziyeti sonucu gelişen, belirli bir bölgeye lokalize, retiküler görünümlü akut dönemde eritendi, kronik dönemde hiperpigmente yamalar ile ka-rakterize bir dermatozdur. Döküntünün nedeni yanık oluşturmayacak şiddetteki bir ısı kaynağına kronik olarak maruz kalınmasıdır. Eski dönemlerde en sık neden soba ile ısınma iken günümüzde sıcak su torbaları, elektrikli ısıtıcılar ve diz üstü bilgisayarlar daha sık neden olmaktadır. Lezyonlar tipik olarak ısı maruziyetinin olduğu bölgede lokalize olma eğilimindedir. Görünüm olarak ayırıcı tanıda livedoid vaskülopati, kütanöz poli-arteritis ııodoza, izole kütanöz vaskiilit, livedo retikiilaris, livedo rasemoza yer almaktadır. Yazımızda livedoid yapıda döküntü nedeniyle vaskülit ön tanısıyla çocuk romatolojiye refere edilen ve eritema ab iğne tamsı konulan iki olgu sunulmuştur. Olgu: 16 yaşında kız olgu son 1.5 aydır her iki alt bacak ön yüzeyde döküntü nedeniyle başvurdu. Fizik muayenede bilateral, diz alundan tibia orta bölümüne uzanan, üzerinde yer yer krudar içeren rctiküler hiperperpigmentasyon (Şekil PS-276a) mevcut idi. Başka bir dcrmatolojik muayene bulgusu olmayan hastanın kollagen doku hastalığı düşündürecek ek muayene bulgusu da yoktu. Tam kan sayımı, akut faz yanıtları ve taasiyonu normal sınırlarda idi. Aynı hafta içinde 15 yaşmda erkek olgu son 3 haftadır her iki alt bacak ön yüzde döküntü nedeniyle başvurdu. Fizik muayenede bilateral, diz altı ile tibia orta kısmına yerleşen livedoid görünümde eritemli, retiküleryama mevcuttu (Şekil PS-276b). İkinci olguda da sistemik hastalık düşündürecek ek bir bulgu yoktu ve laboratuvar incelemeleri ve tansiyonu normal sınırlarda idi. Hastaların hikayesi derinleştirildiğinde ısınmak için elektrikli soba kullandıkları ve ısına önünde oturdukları öğrenildi. Her iki olguda da tekrarlayan ısı maruziyetinin olması, lezyonların sadece direkt ısı teması olan yerlerde olması, sistemik hastalık düşündürecek ek muayene veya laboratuvar bulgularının olmaması nedeniyle eritema ab iğne tanısı konuldu. Lokal tedavi ve ısı maruzi-yetinden uzak durulması önerilen olguların kontrol muayenelerinde lezyonlarda belirgin gerileme olduğu görüldü. Sonuç: Sonuç olarak görünüm olarak romatolojik hastalık döküntülerine çok benzesede belli bir bölgeye lokalize livedoid tarzda döküntüsü olan olgularda tekrarlayan ısı maruziyeti sorgulanmalı ve eritema ab iğne olasılığı akılda tutulmalıdır. [ABSTRACT FROM AUTHOR]

Amaç: Uvea gözün damar ve pigmentten zengin orta tabakasıdır. Üvea tabakasının inflamasyonuna üveit denilir ve tutulan bölümün yerine göre ön, orta, arka ve panüveit olarak isimlendirilir. Üveit nedenleri arasında enfeksiyonlar, non-enfeksiyöz nedenler ve maligniteler yer almaktadır. Non-enfeksiyöz nedenler arasında en sık romatolojik hastalıklar (JIA, Behçet hastalığı, sarkoidoz, vaskülitler), TINU ve idiyopatik üveit yer almaktadır. Çalışmamızın amacı çocuk romatoloji klüıiğimizde non-enfeksiyöz üveit tanısıyla takip ve tedavi edilen hastalarımızın demografik özelliklerini saptamaktır. Yöntem: Çalışmamıza Mayıs 2010 ile Eylül 2017 tarihleri arasında üveit tanısı alan, düzenli kontrollere gelen ve en az 6 ay süre ile kliniğimizden takip edilen hastalar alınmıştır. Bulgular: Çalışmaya 54 jüvenil üveit hastası dahil edilmiştir. Üveit nedeni olarak 27 hasta da (%50) JIA, 6 hastada (%11) Behçet hastalığı, 4 hastada (%7) TINU saptandı. On yedi (%31) hastada idiyopatik üveit tanısıyla takipli idi. Üveit nedeniyle hastalarımızın ortalama takip süresi 18.6 ay (6-60 ay) idi. Cinsiyet dağılımma bakıldığında 28 laz, 26 erkek hasta vardı. JIA olgularında hu oran yine birbirine yalan (15 kız, 12 erkek) idi. idiyopatik üveitte belirgin kız hakimiyeti (11 laz, 6 erkek) var iken, Behçet üveiti (5 erkek, 1 laz) ve TINU da (3 erkek, 1 laz) erkek hakimiyeti mevcuttu. Tüm JIA'larda üveit görülme sıklığı %6.6 (27/405) iken bu oran Behçet hastalarında %22.2 (6/27) olarak bulundu. JIA hastalarının 18'i (%67) persistan oli-goJIA idi. Beş hastada JIA taıusı konulduğu anda (4 persistan oligoJIA, 1 RF negatif poliJIA) üveit saptandı. JIA hastalarında JIA tanısı ile ilk üveit saptanması arasındaki ortalama süre 21.2 (0-77) ay idi. Üveit tanısı konulduğunda ortalama yaş 9.1 yıl (1-17.5) iken, persistan oligoJIA 1ar da ortalama 5, idiyopatik üveitte 11.8, Behçet hastalarında 13.7 ve ERA'larda 16.6 yaş olarak bulundu. Çalışmaya alınma anında 45 hasta remisyonda iken 9 hastanın göz bulguları aktif idi. İdiyopatik üveitte 5 hasta, JLA grubunda 2 hasta, Behçet ve TINU'da birer hastanın göz bulguları remisyonda değildi. JIA'larda metotreksat dirençli üveit nedeniyle biyolojik ajan 14 hastada (%52; adalimumab 11 hasta, tosilizumab 3 hasta) kullanıldı. Sonuç: Erken taıu ve tedavi ile komplikasyon gelişimi engellenebileceği içüı JIA ve Behçet hastalarında düzenli aralıklarla üveit açısından detaylı göz muayenesi yapılması önemlidir. [ABSTRACT FROM AUTHOR]

Objective: The objectives of this study were to determine the musculoskeletal (MSK) conditions associated with pediatric psoriasis (Pso) and to evaluate the thickness of Achilles tendon of children with Pso and healthy controls (HCs)., Methods: Pso patients who were followed-up in dermatology outpatient clinic were referred to a pediatric rheumatology center. All patients and healthy peers were evaluated with standardized forms. Both patients and controls underwent ultrasonographic evaluation for Achilles tendon thickness., Results: A total of 55 pediatric Pso and 46 healthy children were included in the study. Of patients with Pso 56.4% had arthralgia, 25.5% had lower back pain, 18.2% had heel pain, 12.7% had hip pain, and 10.9% described morning stiffness. Arthritis was detected in 7.3%, sacroiliac tenderness in 12.7%, and enthesitis in 9.1% of the patients. Arthralgia, lower back pain, and heel pain were significantly frequent in Pso group than healthy children median left and right Achilles tendon thicknesses of Pso patients who were significantly greater than that of HCs prevalence of psoriatic arthritis (PsA) among Pso patients was 7.3%., Conclusion: Evaluation of a child with Pso regularly for the MSK complaints is critical for the early recognition of PsA. Ultrasonography is a useful technique for screening Pso patients for early detection of enthesopaty., Competing Interests: No conflict of interest was declared by the authors., (© Copyright 2023 by Istanbul Provincial Directorate of Health.)

Objective: Juvenile idiopathic arthritis (JIA) is the most common cause of chronic arthritis in children. Biologics have changed the faith of children with rheumatic diseases. The main objective of this study was to demonstrate the rate of usage, efficacy and safety of biologics in JIA subtypes., Methods: This retrospective observational cohort study was conducted between May 2010 and September 2017. All children with the diagnosis of JIA and children under a biological agent treatment were recorded into the local registry system. Age, gender, JIA subtype, medications used, the clinical status of the patient, tuberculosis screening results, and side effects observed under biologics were retrieved from the registry., Results: There were 405 patients with the diagnosis of JIA in the cohort. Biologics were used in 123 (30.3%) JIA patients. Subtype frequencies of JIA patients were as follows: persistent oligoarticular JIA (33.6%), enthesitis-related arthritis (29.2%), systemic JIA (13%), rheumatoid factor (RF)-negative polyarticular JIA (13%), extended oligoarticular JIA (4.2%), RF-positive polyarticular JIA (3.4%), psoriatic arthritis (1.8%) and unclassified arthritis (1.8%). The rate of biologic use was high in extended oligoarticular JIA (64.7% of the cases), RF-positive polyarticular JIA (57.1%), psoriatic arthritis (57.1%), RF-negative polyarticular JIA (41.5%), and in systemic JIA (39.6%). Enthesitis-related arthritis (27.1%), persistent oligoarticular JIA (17.6%) and unclassified arthritis (16.6%) patients were the cases that needed a biologic agent in the last order. At the last control, 78.9% of the cases were in remission, while 21.1% of them were active despite biologic treatment. Isoniazid prophylaxis was used in 30.8% of the patients. None of the patients developed active tuberculosis infection under prophylaxis. Adverse events were observed in 18.6% of patients under biologics as recurrent uncomplicated upper respiratory tract infections being the most common., Conclusion: Biologics are safe and effective treatment options in children with JIA. Most of the JIA patients with polyarticular involvement require biologics earlier in the disease course. The risk of tuberculosis infection seems not to be increased after appropriate screening and prophylaxis., Competing Interests: Conflict of Interest: No conflict of interest was declared by the authors., (Copyright: © 2020 by Istanbul Northern Anatolian Association of Public Hospitals.)

Background: Transient tachypnea of the newborn (TTN) is the most common cause of respiratory distress in newborns. Although associated with some morbidity, it is generally believed that once TTN resolves, there is no further increased risk for respiratory disease. However, in limited studies frequency of wheezing attacks is found to be increased in patients who had TTN diagnosis during the newborn period, in comparison to patients who had no respiratory problem. Thus, the question arises as to whether TTN is an innocent disease., Methods: This study was done retrospectively. We recorded the demographic characteristics of 103 infants born between 17 October 2003 and 17 October 2004 at Zeynep Kamil Hospital and hospitalized because of TTN in the neonatal intensive care unit. In the second phase, we telephoned the parents of the 103 infants and asked about wheezing attacks. A total of 103 other infants, born during the same period, with no health problems during the newborn period, were included in the study as the control group and the same procedures were applied to them., Results: The rate of wheezing attack among patients with TTN diagnosis was found to be significantly higher than that in patients who had no TTN diagnosis (P < 0.01). TTN was found to be an independent risk factor for wheezing attack (OR, 2.378; 95% CI, 1.20-4.70)., Conclusion: In conclusion, we established that TTN is an independent risk factor for wheezing. In addition we also hypothesized that genetic and environmental interactions synergistically predisposed these children for future wheezing., (© 2011 The Authors. Pediatrics International © 2011 Japan Pediatric Society.)

Abstract Background: This study compared the rates of decrease in serum bilirubin levels in severely jaundiced healthy term infants given oral or intravenous fluid supplementation during phototherapy. Methods: A randomized controlled study was carried out in the neonatal intensive care unit of Zeynep Kamil Maternity and Children Hospital (Istanbul, Turkey) over a 4-month period. Two hundred fifty healthy term infants with hyperbilirubinemia were randomized to receive either solely breastmilk (n=125) or both breastmilk and intravenous fluid (n=125) during phototherapy. Results: There were no significant differences (p>0.05) in the mean birth weight, mean gestastional age, modes of delivery, mean time of admission age, mean serum osmolality, and hematocrit and reticulocyte count between the two groups. Similarly, there was no significant difference (p>0.05) in the mean indirect serum bilirubin level at the time of admission to the neonatal intensive care unit and at 4, 8, 12, 24, and 48 hours after commencement of phototherapy between the two groups. There was no significant difference (p>0.05) in the mean duration of phototherapy or in the median duration of hospitalization between the two groups. Conclusions: Based on our results, intravenous fluid support has no effect on the rate of decrease in serum bilirubin and decrease in duration of phototherapy in healthy term newborns with no dehydratation. However, using the oral route avoided the need for intravenous cannulae and their attendant complications. Insensible fluid loss is increased during phototherapy, so protection of hydration status with oral feeding is important for newborns.