A clinical overview of paediatric sarcoidosis: Multicentre experience from Turkey.

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.

Vaccination coverage of children with rheumatic diseases compared with healthy controls: a retrospective case-control study.

OBJECTIVE: To reveal the vaccination status of patients with pediatric rheumatic disease (PedRD) and to compare this with healthy controls. METHODS: The electronic health records of the Ministry of Health regarding the vaccination status of children with PedRD followed in a tertiary hospital were analyzed cross-sectionally and compared with their healthy controls. The missing vaccines were reported according to individual, age-appropriate schedule and causes of skipped vaccines in both groups were investigated with an online survey. RESULTS: The vaccination rate of patients in the last examination was 71.4% (90/126) and 95.7% (110/115) in healthy controls (p < 0.001). Measles-mumps-rubella vaccine, diphtheria, the administration rates of the second dose of tetanus-acellular pertussis-inactivated polio and Haemophilus influenzae type B, chickenpox, and hepatitis A vaccines were significantly lower in patients than in controls (p values 0.004, 0.02, 0.01, 0.013, respectively). The pre-diagnosis incomplete vaccination proportion was significantly higher in the patient group (16.6%) than in healthy controls (4.3%) (p = 0.002). In the patient group, the proportion of incomplete live-attenuated vaccines after diagnosis (25%) was more than pre-diagnosis (61.1%) (p = 0.04), while the proportion of incomplete non-live vaccines before and after diagnosis was similar (47.2% and 50%, respectively) (p = 0.73). The major reasons for missed vaccines were physicians' recommendations (15.6%), the presence of PedRD diagnosis (12.5%), and the drugs used (12.5%). CONCLUSION: Vaccination coverage of PedRD patients has been shown to lag behind the routine vaccination schedule (71.4%). In addition to new recommendations, electronic health system records for vaccination may be appropriate for the follow-up of these patients, and the addition of reminder alerts may be useful to reduce the rate of missed vaccinations.

Adherence to best practice consensus guidelines for familial Mediterranean fever: a modified Delphi study among paediatric rheumatologists in Turkey.

BACKGROUND: Although not validated fully, recommendations are present for diagnosis, screening and treatment modalities of patients with familial Mediterranean fever (FMF). OBJECTIVE: To review the current practices of clinicians regarding FMF and reveal their adherence to consensus guidelines. METHODS: Fifteen key points selected regarding the diagnosis and management of FMF were assessed by 14 paediatric rheumatologists with a three-round modified Delphi panel. RESULTS: Consensus was reached on the following aspects: genetic analysis should be ordered to all patients when clinical findings support FMF, but its result is not decisive alone. In the absence of clinical features, colchicine should be commenced when two pathogenic alleles and family history of amyloidosis are present. Serum amyloid A testing at each visit is recommended in patients resistant to colchicine, with subclinical inflammation and family history of amyloidosis. Consensus was reached on both the definition of colchicine resistance and starting biologic in resistant cases. Cost, efficiency, ease of use, treatment adherence, accessibility and emergence of adverse events are the factors affecting the choice of biologic agents. In patients without any attack and evidence of subclinical inflammation within the last 6 months following initiation of biologics, treatment dose intervals can be prolonged. CONCLUSION: A consensus was achieved regarding the routine diagnosis and screening and treatment of FMF patients. The definition of colchicine resistance was made and a protocol was created for prolongation of treatment intervals of biologic agents. We anticipate that the results of the study reveal real-life data on the approach to patients in clinical practice.

Shear wave elastography evaluation of liver, pancreas, spleen and kidneys in patients with familial mediterranean fever and amyloidosis.

OBJECTIVES: Amyloid deposits in a visceral organ can contribute to tissue stiffness that could be measured with shear wave elastography (SWE). We aimed to investigate changes in organ stiffness in conjunction with laboratory parameters in patients with Familial Mediterranean Fever (FMF) and amyloidosis. METHODS: This prospective study included 27 FMF patients, 11 patients with amyloidosis, and 38 healthy controls. Median shear wave elasticity values of the liver, spleen, both kidneys, and pancreas on SWE were compared among study and control groups. The mean values of CRP (C-reactive protein) and ESR (erythrocyte sedimentation rate) were compared by the t-test and the median of SAA (serum amyloid A protein) was compared with the Mann-Whitney U test between FMF groups with and without amyloidosis. Spearman's correlation analysis was performed to reveal the association between stiffness values and laboratory parameters. RESULTS: The median liver, spleen, kidney, and pancreas elasticity values were significantly higher in the FMF group with amyloidosis compared to control subjects. The median kidney stiffness values in the FMF group with or without amyloidosis were significantly higher compared to control subjects. Median liver stiffness values in FMF patients with amyloidosis were significantly higher than FMF patients without amyloidosis. There were statistically significant positive correlations between the CRP (p = 0.001, r = 0.56), ESR (p = 0.001, r = 0.61), and SAA (p = 0.002, r = 0.53) levels with spleen stiffness, and CRP (p = 0.006, r = 0.48) and ESR (p = 0.001,r = 0.61) levels with pancreas stiffness, and ESR (p = 0.004, r = 0.51) levels with the left kidney stiffness. CONCLUSION: SWE could be a potential tool for noninvasive follow-up of FMF patients and also amyloid deposition. ADVANCES IN KNOWLEDGE: Both acute inflammation and amyloidosis in the FMF patients could increase organ stiffness.

A controversial topic in juvenile idiopathic arthritis: Association between biologic agents and malignancy.

OBJECTIVE: Over the last 2 decades, the usage of biological agents in the treatment of juvenile idiopathic arthritis (JIA) has been a successful and promising approach in controlling disease activity and preventing chronic sequelae. However, there are ongoing concerns about the long-term safety data and side-effect profile. We aimed to present preliminary data on the incidence of malignancy in patients with JIA treated with biological agents versus the general population rates in Turkey. METHOD: A single-center hospital-based cohort study was performed to analyze cancer occurrence among JIA patients treated with biologic agents during the observation period between January 2004 and May 2019. As reference data for direct standardization, age, gender, and calendar year-specific incidence rates from the Turkish cancer registry were used. The standardized incidence ratio (SIR, ratio of cancers observed to expected) was generated with 95% confidence intervals. RESULTS: The study sample consisted of 1023 JIA patients who had been treated with biologic or non-biologic agents. In the biologic-experienced group (n = 656), the mean age (at the study) was 16.7 ± 5.6 years. The mean length of follow-up was 9.9 ± 5.0 years. One cancer was detected within the observation period (SIR: 1.3, 95% CI: 0.06-6.3). The patient was an 18-year-old male who had previously received etanercept and tocilizumab until the diagnosis of the hematologic malignancy (SIR: 2.5, 95% CI: 0.1-12.6). CONCLUSION: Patients treated with biologic agents appeared to have an increased rate of incident hematologic malignancy versus the general population in Turkey. However, before mentioning a clear causal relationship, other potential contributing factors should be taken into consideration.

Echogenic lymph nodes in the differential diagnosis of pediatric sarcoidosis.

We present a delayed diagnosis of sarcoidosis in an 11-year-old girl by demonstrating ultrasonographic imaging findings of granulomatous cervical and abdominal lymph node involvement. Pulmonary interstitial fibrosis in addition to multi-compartmental enlarged echogenic lymph nodes could be considered sarcoidosis. Punctate echogenic foci in the cervical lymph nodes should be considered in the differential diagnosis of sarcoidosis.

The Assessment of Serum Endocan Levels in Children With Juvenile Idiopathic Arthritis.

OBJECTIVES: This study aims to evaluate the levels of serum endocan in children with juvenile idiopathic arthritis (JIA). PATIENTS AND METHODS: Sixty-seven children with JIA (30 males, 37 females; mean age 10.4±4.9 years; range 2 to 18 years) and a sex- and age- matched healthy control group of 39 children (16 males, 23 females; mean age 9.3±4.1 years; range 1 to 17 years) were recruited. Patients with JIA were divided into two groups as the clinically active JIA group (n=27) and inactive JIA group (n=40). RESULTS: The median serum endocan level in patients with JIA was significantly higher than in the control group (633.75 ng/L vs. 379.76 ng/L, p<0.01). Comparison between patients with active JIA and inactive JIA was not significant in terms of endocan levels (618.70 ng/L vs. 687.36 ng/L, p=0.34). There was a weak negative correlation between Childhood Health Assessment Questionnaire scores of patients with JIA and serum endocan levels. CONCLUSION: The high level of serum endocan highlighted the endothelial damage in patients with JIA.

Evaluación del lipidograma y ciertos factores de riesgo de ateroesclerosis en niños cuyos padres tuvieron arteriopatía coronaria de inicio temprano / Assessment of lipid profile and some risk factors of atherosclerosis in children whose parents had early onset coronary artery disease

Antecedentes/Objetivo. El objetivo de nuestro estudio fue analizar el lipidograma y ciertos factores de riesgo de ateroesclerosis, tales como las lipoproteínas de baja densidad oxidadas (ox-LDL, por su sigla en inglés) y las lipoproteínas de baja densidad pequeñas y densas (sdLDL, por su sigla en inglés) en los hijos de pacientes con cardiopatía coronaria (CC) prematura. Población y métodos. Hijos de padres con CC de inicio temprano emparejados con pares de su misma edad y mismo sexo. Se analizaron las concentraciones de lípidos, apolipoproteínas (ApoA, B, E), ox-LDL, sdLDL y lipoproteína (a) [Lp(a)] en los niños de estudio y de referencia. Los datos se evaluaron con el programa SPSS, junto con la prueba t de Student y la prueba U de Mann-Whitney. Resultados. Los niños del grupo de estudio (n: 43) tenían niveles más elevados de LDL, Lp(a) y ox-LDL y cocientes mayores de CT/HDL, ApoB/ApoA, LDL/HDL y ox-LDL/HDL (p < 0,05) que los del grupo de referencia. Conclusión. Con base en estos hallazgos, se sugiere que la dislipidemia y las concentraciones elevadas de LDL, Lp(a) y ox-LDL son frecuentes en los hijos de pacientes con CC de inicio temprano y representan gran parte de la predisposición familiar a tener CC

Background/Aim: The objective of our study was to analyze the lipid profile and some risk factors of atherosclerosis such as oxidized-low density lipoprotein (ox-LDL), small dense LDL (sd LDL) in the offspring of patients with premature coronary heart disease (CHD). Population and Methods: Children whose parents had early onset CHD were matched with age and sex pairs. Study and controls were analyzed for lipid levels, apolipoproteins (Apo- A,B,E), ox-LDL, sd LDL and lipoprotein (a) [Lp(a)]. The data were evaluated with SPSS using "Student tand Mann-Whitney U" tests. Results: Thestudy group children (n: 43) had higher LDL, Lp(a) and ox-LDL levels, ratios of TC/HDL, Apo-B/A, LDL/HDL and ox-LDL/HDL (p<0.05) than control group. Conclusion: These findings suggest that dyslipidemia and increased LDL, Lp(a) and ox-LDL levels are common in the offspring of patients with early onset CHD and account largely for their familial predisposition for CHD.

Assessment of lipid profile and some risk factors of atherosclerosis in children whose parents had early onset coronary artery disease. / Evaluación del lipidograma y ciertos factores de riesgo de ateroesclerosis en niños cuyos padres tuvieron arteriopatía coronaria de inicio temprano.

BACKGROUND/AIM: The objective of our study was to analyze the lipid profile and some risk factors of atherosclerosis such as oxidized-low density lipoprotein (ox-LDL), small dense LDL (sd LDL) in the offspring of patients with premature coronary heart disease (CHD). POPULATION AND METHODS: Children whose parents had early onset CHD were matched with age and sex pairs. Study and controls were analyzed for lipid levels, apolipoproteins (Apo- A,B,E), ox-LDL, sd LDL and lipoprotein (a) [Lp(a)]. The data were evaluated with SPSS using "Student t and Mann-Whitney U" tests. RESULTS: The study group children (n: 43) had higher LDL, Lp(a) and ox-LDL levels, ratios of TC/HDL, Apo-B/A, LDL/HDL and ox-LDL/HDL (p<0.05) than control group. CONCLUSION: These findings suggest that dyslipidemia and increased LDL, Lp(a) and ox-LDL levels are common in the offspring of patients with early onset CHD and account largely for their familial predisposition for CHD.

Antecedentes/Objetivo. El objetivo de nuestro estudio fue analizar el lipidograma y ciertos factores de riesgo de ateroesclerosis, tales como las lipoproteínas de baja densidad oxidadas (ox-LDL, por su sigla en inglés) y las lipoproteínas de baja densidad pequeñas y densas (sdLDL, por su sigla en inglés) en los hijos de pacientes con cardiopatía coronaria (CC) prematura. Población y métodos. Hijos de padres con CC de inicio temprano emparejados con pares de su misma edad y mismo sexo. Se analizaron las concentraciones de lípidos, apolipoproteínas (ApoA, B, E), ox-LDL, sdLDL y lipoproteína (a) [Lp(a)] en los niños de estudio y de referencia. Los datos se evaluaron con el programa SPSS, junto con la prueba t de Student y la prueba U de Mann-Whitney. Resultados. Los niños del grupo de estudio (n: 43) tenían niveles más elevados de LDL, Lp(a) y ox-LDL y cocientes mayores de CT/HDL, ApoB/ApoA, LDL/HDL y ox-LDL/HDL (p < 0,05) que los del grupo de referencia. Conclusión. Con base en estos hallazgos, se sugiere que la dislipidemia y las concentraciones elevadas de LDL, Lp(a) y ox-LDL son frecuentes en los hijos de pacientes con CC de inicio temprano y representan gran parte de la predisposición familiar a tener CC

[The comparison a 16-year follow-up results of balloon angioplasty for aortic coarctation in children of different age groups: a single-center experience]. / Aort koarktasyonu tanisiyla balon anjiyoplasti uygulanan farkli yas gruplarindaki çocuklarin 16 yillik izlem sonuçlarinin karsilastirilmasi: Tek merkez deneyimi.

OBJECTIVE: Pediatric patients with different age groups who underwent balloon angioplasty for aortic coarctation were evaluated for recoarctation, aneurysm, peripheral arterial injuries and concomitant diseases. METHODS: From January 1994 to 2010, 80 patients with aortic coarctation (native/recoarctation) were evaluated, retrospectively. According to age at angioplasty, patients were divided into three groups: Group A (0-3 months, n=29, 25 male/4 female, average weight 4±1.2 kg), Group B (3-12 months, n=20, 15 male/5 girls, average weight 6.5±1.9 kg) and Group C (> 1 year, n= 31, 15 male/16 girls, average weight 22.8±16 kg). The patients were followed with echocardiography and clinical signs. The data of the native and recoarcted patients and also those in three different age groups were analyzed by using Chi-square, Kruskal-Wallis, and Student t tests. RESULTS: Peak systolic pressure gradient was reduced from 42±17 mmHg to 6.2±6 mmHg after balloon angioplasty (p<0.001) [n=80, 56 (70%) native, 24 (30%) recoarctation]. There was no difference between groups for early success. None of them did require immediate surgery. There were ventricular septal defect in 23 (28.7%), bicuspid aorta in 18 (22.5%), patent ductus arteriosus in 11 (13.7%) patients. Two patients had Turner's syndrome. Mean follow-up period was 74±56 months. While recoarctation developed in 20 (25%), [12 (60%) in Group A, 5 (25%) in Group B and 3 (15%) in group C, incidence was higher in the 0-3 months age group (p=0.018). Femoral artery occlusion and aneurysm were developed in 6 (7.5%) and 4 (5%) patients, respectively, and all of them were under 1-year-old. CONCLUSION: Balloon angioplasty can be used a method in treatment of native aortic coarctation and postoperative restenosis. Especially, children under 3 months should be monitored closely after the procedure for recoarctation, aneurysms and peripheral artery problems.

The investigation of prooxidant-antioxidant balance in serum of children with family histories of essential hypertension.

It has been accepted that essential hypertension is associated with a loss of the balance between prooxidation and antioxidation. Thus, excessive oxygen free radical production may be an early event in the pathogenesis of hypertension. To compare lipid peroxidation and antioxidant system in serum of children of essential hypertensive and normotensive parents. Serum malondialdehyde (MDA) levels were measured spectrofluorometrically. Antioxidant activity, glutathione peroxidase, selenium levels in serum were measured as indices of antioxidant power. The peroxidation of apo B containing lipoproteins (VLDL+LDL) was measured as the susceptibility to oxidation in vitro. Serum MDA levels increased, but no marked differences in total antioxidant activity, glutathione peroxidase activity, selenium levels and VLDL+LDL oxidation were found in serum of children with family histories of essential hypertension as compared to children of normotensive parents.