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Objectives: This study aimed to investigate coronavirus disease 2019 (COVID-19) vaccination rates and factors affecting vaccination in children with rheumatic diseases. Patients and methods: This multicenter cross-sectional survey-based study was conducted between July 2022 and September 2022. Four hundred seventy-four patients (256 females, 218 males; median age: 15 years; interquartile range, 13 to 16 years) were included in the patient group, and 211 healthy children (124 females, 87 males; median age: 15 years; interquartile range, 13 to 16 years) were included in the control group. A questionnaire was administered to the parents face-to-face during routine outpatient visits. Results: Of the patients, 220 were followed up with the diagnosis of autoinflammatory disease, 174 with juvenile idiopathic arthritis, 48 with connective tissue disease, 23 with vasculitis, eight with uveitis, and one with sarcoidosis. In the study group, 256 (54%) patients and 115 (54.5%) healthy children received at least one dose of COVID-19 vaccine. Parents' concern regarding potential side effects of the vaccine was the most common reason for COVID-19 vaccination hesitancy in both groups. The median patient age, follow-up period, colchicine treatment rates, childhood vaccination and influenza vaccination rates, median parental age, parental vaccination rate, and parental education level were higher in vaccinated patients (p<0.05 for all). In addition, vaccination rates were high in patients who shared their concerns about vaccination with the rheumatology team (p<0.001). Conclusion: Parents' concerns about safety and side effects were found to be the most important factors affecting vaccination success. Identification of the underlying causes of parental vaccine hesitancy will facilitate the development of effective vaccination strategies for potential future outbreaks. [ABSTRACT FROM AUTHOR]

Background: The aim of this study was to evaluate the role of the systemic immune inflammation index (SII), C‐reactive protein/albumin ratio (CAR), the monocyte/lymphocyte ratio (MLR), and the neutrophil/lymphocyte ratio (NLR) in predicting disease severity, treatment, and prognosis in multisystem inflammatory syndrome in children (MIS‐C). Methods: This medical record review retrospectively evaluated the clinical and laboratory findings of 191 MIS‐C patients followed in the Department of Pediatric Rheumatology at Ankara City Hospital, Turkey. The patients were grouped by disease severity: mild, moderate, and severe. SII, CAR, MLR, and NLR were calculated for each group. Results: All patients had fever at the time of admission; 153 (80.1%) had gastrointestinal tract involvement, 74 (38.7%) had rash, 63 (33%) had conjunctivitis, 107 (56%) had cardiac involvement, 32 (15.6%) had renal involvement, and 143 (74.9%) had hematological involvement. According to logistic regression analysis, SII, NLR, MLR, and CAR were found to be predictive indexes for disease severity, need for intensive care, need for inotropes, and anakinra treatment in MIS‐C. The cut‐off values of ≥1605.3 for SII, ≥9.1 for NLR, and ≥3.9 for CAR increased the risk of severe disease by 3.4, 7.1, and 5.7 times, respectively. Conclusion: NLR, SII, MLR, and CAR are effective and useful for predicting the severity of MIS‐C, the need for intensive care, and the need for anakinra treatment. [ABSTRACT FROM AUTHOR]

Objectives: The aims of this study were to describe the clinical features, comorbidities and outcome of systemic childhood polyarteritis nodosa (PAN) and to evaluate PAN-like diseases in differential diagnosis. Methods: The study group consisted of patients who were diagnosed as PAN in a referral center in Turkey. The files of all patients were reviewed retrospectively. Disease activity was evaluated with pediatric vasculitis activity score (PVAS). Results: A total of 19 (13 boys/six girls) patients were enrolled in the study. The mean age of patients was 10.37 ± 3.6 years. The mean duration of follow-up was 5.73 ± 3.74 years. Eight patients (42.1%) were also diagnosed with familial Mediterranean fever (FMF). The cutaneous involvement was higher in patients with PAN than those with FMF-associated PAN (p¼.03). The median (min-max) PVAS at diagnosis was 5 (3-7). There was no correlation between PVAS scores at the time of diagnosis and age, clinical findings and relapse. CECR1 mutation was detected in one patient leading to deficiency of adenosine deaminase 2. Conclusion: The clinical presentation is variable in children with PAN. PAN-like diseases characterized by necrotizing vasculitis should be considered. The possibility of FMF should be kept in mind if inflammation cannot be controlled. [ABSTRACT FROM AUTHOR]

The diagnosis of Familial Mediterranean fever (FMF) based on clinical findings supported by genetic mutation. Recently, the new Eurofever/PRINTO classification criteria including genetic analysis were established. The aim of this study is to evaluate the performance of the new criteria in FMF patients with a single exon 10 mutation in childhood. The study group consisted of FMF patients who had a single exon 10 mutation in a referral center in Turkey. Patients with periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome were included as a control group. The medical charts of all patients were reviewed retrospectively. A total of 106 FMF patients (59 boys) were enrolled in the study group. The median age at first symptom was 5; the median age at diagnosis was 7 years. The mean follow-up was 33 ± 35.4 months. Majority of the patients (n = 58, 54.7%) had heterozygous M694V, 16 (15%) patients had M694V/E148Q and 13 (13.8%) patients had heterozygous M680I mutation. The sensitivity of the Yalcinkaya-Ozen criteria was 98.1% and it was 97.1% for the Eurofever/PRINTO classification criteria. The specificity of the new Eurofever/PRINTO classification criteria was 96.7% and it was 74.1% for the Yalcinkaya-Ozen criteria. The new Eurofever/PRINTO classification criteria have a good sensitivity as the Yalcinkaya-Ozen criteria in patients with a single exon 10 mutation. Additionaly, the new criteria have better specificity. It should be useful to apply the clinical only criteria where the carrier rate is high. [ABSTRACT FROM AUTHOR]

Objectives: This study aims to investigate the relationship between the Childhood Health Assessment Questionnaire (CHAQ) and types of juvenile idiopathic arthritis (JIA), the disease activity, laboratory findings, and treatments of patients with JIA. Patients and methods: Eighty-two children with JIA (37 males, 45 females; mean age 13.96±4.45 years) and 68 healthy children (31 males, 37 females; mean age 10.1±4.24 years) participated in the study. CHAQ, composed of discomfort and disability indexes, was applied to patients and the control group. Results: CHAQ score was significantly higher in JIA patients than in the control group (p<0.05). Patients having active joint involvement had significantly higher visual analog scale and CHAQ scores than those without active joint involvement (p<0.05). There were no statistical differences between the disease types and CHAQ scores (p>0.05). Visual analog scale pain scores and CHAQ scores were significantly higher in patients in an active disease period than patients in remission (p<0.05). Conclusion: The CHAQ is a reliable method for JIA follow-up. CHAQ clinically discriminates between healthy groups and JIA patients with a high disability index. [ABSTRACT FROM AUTHOR]

Recently, a new set of criteria was established for the diagnosis of familial Mediterranean fever (FMF) in childhood. The aim of this study is to validate the new criteria set among heterozygous patients with clinical features of FMF. The study group consisted of FMF patients, who had a mutation at a single allele, who were followed in four pediatric nephrology-rheumatology centers in Turkey. Patients were evaluated by the new criteria set and also by the Tel Hashomer criteria. According to the new criteria, the diagnosis of FMF was established by the presence of two or more of five criteria (fever, abdominal pain, chest pain, arthritis, family history of FMF). The study group consisted of 110 FMF (54 male, 56 female) patients. Majority of the patients had heterozygous pM694V mutation (65%). The sensitivity of the new criteria set and that of the Tel Hashomer criteria in our study group were found to be 93% and 100%, respectively. In conclusion, this study designates that sensitivity of the new criteria set is also high in patients who had a mutation at a single allele. [ABSTRACT FROM AUTHOR]

Purpose: To determine the indications for penetrating keratoplasty. (PK) at Haydarpasa Numune Training and Researching Hospital Eye Bank,İstanbul, Turkey. Materials and Methods: Retrospectively, we reviewed the records of all patients who underwent PKP at Haydarpasa Numune Training and ResearchingHospital's Eye between 2001and 2007. Results: A total of 303 patients (303 eyes) were included in this study. The leading indications for PKP were keratoconus (23.8%), followed by pseudophakic bullous keratopathy (23.4%), corneal scarring secondary to infectious keratitis (17.2%), traumatic corneal scarring (14.9%), corneal dystrophy (7.6%), regrafting (5.3%), aphakic bullous keratopathy (2.6%), band keratopathy (2.3%), and Fuchs' endothelial dystrophy (1.3%). There was an increasing trend in rates of pseudophakic bullous keratopathy, regrafting, and traumatic corneal scarring over the second half of this period. There was no significiant change in the indication rate of keratoconus, corneal scarring secondary to infectious keratitis and corneal dystrophy. Conclusions: Keratoconus and pseudophakic bullous keratopathy were the most common indications of PK in our eye bank. [ABSTRACT FROM AUTHOR]

Background/objective: Familial Mediterranean fever (FMF) is an autosomal recessive disease, characterized by recurrent, self-limited attacks of fever with serositis. Various diseases were reported to be associated with FMF. The aim of this study was to investigate the frequency and characteristics of sacroiliitis in children with FMF., Methods: Files of FMF patients who had been seen in 2 reference hospitals in Ankara were retrospectively evaluated. Patients with FMF and concomitant sacroiliitis were included to the study. All patients had magnetic resonance imaging evidence of sacroiliitis., Results: Among 650 FMF patients, 17 (11 females, 6 males; mean age, 13.32 ± 4.24 years) (2.6%) of them were found to have sacroiliitis. Familial Mediterranean fever diagnosis was done prior to sacroiliitis diagnosis in 11 patients (65%) and concurrently or afterward in 6 patients (35%). Ten patients had isolated sacroiliitis, and 7 had associated diseases (5 enthesitis-related arthritis, 1 psoriatic arthritis, and 1 ulcerative colitis). Arthritis (59%), arthralgia (77%), leg pain (71%), heel pain (41%), and enthesitis (29%) were common complaints. Sacroiliac tenderness was detected in 77%, and M694V mutation in almost 90% of the patients. All patients received colchicine therapy. Additionally, 14 of them were treated with nonsteroidal anti-inflammatory drugs, 10 were on sulfasalazine treatment, and 7 of them were on biological agents., Conclusions: Sacroiliitis can be seen in patients with FMF during childhood, and M694V mutation seems to be a susceptibility factor for its development. Inflammatory low-back pain and leg and heel pain could suggest sacroiliitis.

Background: Despite constant improvements in caring for critically ill neonates and infants with congenital cardiac disease, sepsis, bone marrow and solid organ transplantation, acute renal failure (ARF) is an important problem in these children. ARF, severe fluid overload and inborn errors of metabolism are some of the indications for acute dialysis in infants and children., Methods: The authors had retrospectively evaluated the medical records of Pediatric Intensive Care Unit, Ankara University School of Medicine, Ankara, Turkey patients who had required acute renal replacement therapy between the dates of January 2002 to February 2005., Results: Medical records of 332 patients were reviewed. Acute renal replacement therapy was performed in 21 patients (6.3%; mean age, 9.6 +/- 7.4 years). Dialysis modalities were peritoneal dialysis in 15 patients (71.4%; mean age, 3.9 +/- 5.6 years) and hemodialysis in six patients (28.6%; mean age, 12.1 +/- 3.2 years). A total of 90% of patients had severe systemic disease leading to ARF. A total of 95% of patients had multiple organ dysfunction syndrome. The most common cause of ARF was refractory shock. At the beginning of renal replacement therapy, 10 patients were anuric, nine patients had volume overload, seven patients had decompensated metabolic acidosis and nine patients had hypotension. The average dialysis period was 4.7 +/- 6.4 days. Mortality rate was 66.7%. Eight patients recovered from ARF and chronic renal failure had developed in one patient., Conclusion: In the Pediatric Intensive Care Unit, ARF is frequently seen together with multiple organ dysfunction syndrome and the mortality rate is high. Both peritoneal dialysis and hemodialysis are important renal replacement treatment modalities in patients with ARF. The age and hemodynamic status of the patients are important when choosing treatment modality; generally peritoneal dialysis is preferred in infants and toddler, while hemodialysis is preferred in older children.

The changing pattern of antimicrobial resistance in the causative microorganisms of urinary tract infection (UTI) in childhood is a growing problem. The aims of this study were to assess the resistance patterns of urinary isolates to commonly used antimicrobials and to evaluate the options for empirical treatment of UTI. A prospective cross-sectional analysis of bacteria isolated from children with UTI was performed between January 2003 and January 2004. Resistance to antibiotics was analysed in three age groups: Group I, < or =12 months; Group II, 13-60 months; and Group III, >60 months. A total of 165 urinary pathogens were isolated from 131 patients. Mean patient age was 63.7+/-49.8 months. The most common causative agent was Escherichia coli (87% of cases) followed by Klebsiella pneumoniae (10%). Resistance to ampicillin (74.2%) and co-trimoxazole (61.3%) was significant in all isolates. Nitrofurantoin was the most active agent against E. coli (2.2% resistant isolates), followed by amikacin (4.9%), ceftriaxone (7.5%) and ciprofloxacin (12%). None of the isolates from Group I patients were resistant to ciprofloxacin and a low resistance rate (7.1%) was noted for amikacin. In Group II patients, none of the isolates were resistant to amikacin, and ceftriaxone was the second most suitable antibiotic (resistance rate 2.2%). In Group III patients, the lowest resistance rate was against nitrofurantoin (2.7%). In conclusion, we observed that the use of ampicillin and co-trimoxazole as a single agent for empirical treatment of a suspected UTI would not cover the majority of urinary pathogens in our region. Whilst amikacin, with a negligible resistance rate, was suitable in all age groups, gentamicin might still be useful as an empirical treatment of UTI in children aged >1 year. Nitrofurantoin could be included as a reasonable alternative in the empirical treatment of lower UTI in older children.

Mutations in the NPHS2 gene are a frequent cause of familial and sporadic steroid-resistant nephrotic syndrome (SRNS). Inter-ethnic differences have also been suggested to affect the incidence of these mutations. The frequency and spectrum of podocin mutations in the Turkish population have remained largely unknown. As such, the aim of this study was to screen for podocin mutations in Turkish patients with SRNS. Thirty two patients from 30 unrelated families with SRNS were examined. There were seven familial cases from five different families and 25 sporadic cases. PCR-single-strand conformation polymorphism (SSCP) analysis of the NPHS2 gene was followed by direct sequencing. Five different NPHS2 mutations were detected in four of the 30 (13.3%) families studied; five familial patients from three unrelated families (60%) and one sporadic case (4%) were found to carry podocin mutations. The detected mutations included homozygous c. 419delG, compound heterozygous p. [Arg238Ser] + [Pro118Leu], homozygous p. [Pro20Leu; Arg168His] and heterozygous p. Pro20Leu. Two siblings with compound heterozygous mutations had been reported previously by our group. Podocin mutations were found to be responsible for some of the SRNS cases in Turkey, especially when there was more than one affected person in the family. Our results also suggest the presence of a wide range of phenotypic variability between individuals with the same genotype.

Steroid-resistant nephrotic syndromes often are resistant to additional immunosuppressive agents and tend to progress to end-stage renal disease. Genetic studies in children with familial nephrotic syndrome have identified mutations in genes that encode important podocyte proteins. NPHS2 mutations are responsible for autosomal recessive familial focal segmental glomerulosclerosis (FSGS), and these mutations were detected in both familial and sporadic forms of FSGS. Interethnic differences were suggested to play a role in the incidence of these mutations. In this report, the cases of 3 siblings with steroid-resistant nephrotic syndrome who carry NPHS2 mutations (R238S and P118L) are presented.