Because childhood obesity is still a growing problem in the world, we conducted this study aiming to show the trend in obesity (OB) and overweight (OW) prevalence in the last decade, to observe the alteration of OB and OW prevalence according to age groups and to construct the new age and gender specific body mass index (BMI) reference percentile charts for Turkish children living in the city center of Antalya. u n c o r r e c t e d p r o o f Methods: This cross-sectional study includes 1687 school aged children. International Obesity Task Force (IOTF) references were used to determine the obesity and overweight prevalence. OW was defined as BMI between 85 th and 95 th percentile, and OB above the 95 th percentile. The data obtained were compared with another study carried out by the same unit in the same region in 2003. LMS method, which summarizes terms of three smooth age specific curves called L (lambda), M (mu), and S (sigma), was used to construct the BMI reference percentile charts for Turkish children living in Antalya. Results: The prevalence of OB was 9.8 %, OW was 23.2 % and combined OW/OB was 33 %. Obesity prevalence was higher in boys than girls (p<0.05). The prevalence of combined OW/OB was highest at the age of 9-10 years. The prevalence of obesity has increased 2.9 times during twelve years in Antalya, Turkey. Conclusion: Comparing with the findings of previous decade, childhood obesity in Antalya has reached alarming level. Urgent measures should be taken to prevent obesity and more surveillance studies should be planned to show the future trend of obesity prevalence. What is already known on this topic? Increase in the frequency of obesity and overweight among children and adolescents is a major public health problem all over the world in both developed and developing countries. What this study adds? This is a 12-year interval study of our previous study conducted in 2006. Our findings suggest that OW and OB prevalence in the school-age children living in the same geographical region of Turkey have increased dramatically.
Background:Fabry disease (FD) is a rare metabolic disorder caused by the mutations in the α-galactosidase A (GLA) gene. FD patients present with heterogeneous clinical manifestations, which may overlap with systemic diseases including familial Mediterranean fever (FMF). Recurrent episodes of fever, abdominal pain, and arthralgias can be observed in both disorders and this may lead to misdiagnosis.Objectives:To investigate FD prevalence in mild and severe FMF patients.Methods:A total of 66 FMF patients, according to the Tel-Hashomer criteria, were included in the study. Patients were grouped into mild (Group 1) and severe (Group 2) subsets according to the severity score. α-GLA enzyme activity and mutations in the GLA gene were performed. Demographic features, clinical findings, MEFV mutations and treatments were recorded.Results:The clinical and demographical characteristics of the patients were given in Table 1. In severe form, 27 patients were using biological drug and 40.7% had amyloidosis. Symptoms related to FD including hypohidrosis, acroparesthesias, and painful neuropathies, were not different between the groups. Only one patient in group 1 had a low GLA enzyme activity (0.1 nmol/h/ml;Normal >2.5) which also had mutations in the GLA gene but MEFV mutation test was negative. (Table 2). This patient was a 39-year-old female with recurrent abdominal pain, distal extremity pain and the presence of fever during the attacks. She was heterozygous for R301Q. In detailed history, she reported mild acroparesthesias, hypohidrosis, and tinnitus.Table 1.Demographic and clinical findingsAll patientsn: 66Group 1n: 32Group 2n: 34p-valueAge, median (min./max.)34 (17/64)27 (17/59)36 (18/64)0.192Male, n (%)36 (54.5)14 (43.8)22 (64.7)0.137Disease duration, median (min./max.)20.5 (1/57)12.5 (2/50)25 (1/57)0.006Family history of FMF, n (%)41 (62.1)22 (68.8)19 (57.6)0.443Alpha-galactosidase A (nmol/h/ml), median (min./max.)5.9 (0.1/16)5.6 (0.1/9.6)6 (3.1/16)0.330Abdominal pain, n (%)58 (87.9)31 (96.9)27 (79.4)0.030Fever, n (%)54 (81.8)25 (78.1)29 (85.3)0.532Arthritis, n (%)34 (51.5)10 (31.3)24 (70.6)0.003Pleuritis, n (%)31 (47)19 (59.4)12 (35.3)0.083Painful neuropathy, n (%)23 (34.8)13 (40.6)10 (29.4)0.440Acroparesthesias, n (%)9 (13.6)6 (18.8)3 (8.8)0.240Angiokeratomas, n (%)0 (0)0 (0)0 (0)-Cardiac abnormalities1 (1.5)1 (3,1)0 (0)0.485Tinnitus, n (%)4 (6.1)3 (9.4)1 (2.9)0.274Hearing loss, n (%)2 (3)2 (6.2)00.086Hypohydrozis, n (%)2 (3)1 (3.1)1 (2.9)0.965Cornea verticillata, n (%)0 (0)0 (0)0 (0)-Proteinüria, n (%)13 (19.7)2 (6.3)11 (32.4)0.012Colchine dosing (mg/day), median (min./max.)2 (1/3)1 (1/2)2 (1/3)<0.001Table 2.MEFV mutant alleles and GLA mutationsAll patientsn: 66Group 1n: 32Group 2n: 34Alpha -galactosidase A (GLA) gene mutations, n (%)1 (1.5)1 (3.1)0 (0)M694V mutations, n (%)47 (35.6)17 (26.5)30 (44.1)Non-M694V mutations, n(%)36(27.2)20 (31.2)16 (23.5)Conclusion:In this study, we showed the following: 1) the FD rate in the total FMF group was 1.5% (3.1% in group 1), 2) none of the patients in the severe FMF subset had abnormal enzyme activity or mutations related with FD, 3) symptoms related with FD such as hearing loss, hypohidrosis, acroparesthesias, and painful neuropathies also noted in FMF patients particularly in the milder group. Based on our results, FD should be considered in the differential diagnosis of FMF particularly in patients with atypical symptoms.Disclosure of Interests:None declared
BackgroundAlkaptonuria (AKU) is a metabolic disorder that causes accumulation of oxidized homogentisic acid (HGA) in the connective tissues. The excessive deposition of HGA and its metabolites can cause joint destruction and skeletal abnormalities which the entity is clinically referred as ochronotic arthropathy (OA) [1].ObjectivesTo assess clinical, demographic features and radiographic findings in patients with OA.MethodsAdult AKU patients registered in the database were included in the study. All patients investigated for the presence of OA and inflammatory symptoms. Patients with musculoskeletal symptoms (MSK) underwent conventional X-rays and routine laboratory evaluation. In cases suggestive of inflammatory disease additional work-up such as autoantibodies, HLA-B27 and MRI with inflammatory protocol were performed.ResultsSix out of 15 patients had symptoms compatible with OA (40%; 4 male [M], 2 female [F], median age 56 [51-62] years). The median duration of MSK symptoms were 7 (2-19) years. None of the patients had family history of rheumatologic disease. Baseline CRP were normal in all patients. The HLA-B27 test was negative in all cases. One patient had high titers of rheumatoid factor along with positive anti-CCP that were accompanying erosive arthritis on MCP joints by X-rays. Two patient had positive ANA. All patients had chronic back pain and had changes compatible with OA in their spines (narrowing of the intervertebral spaces, vacuum phenomenon and intervertebral disc calcification). Two patient had inflammatory type of pain character (IBP). Radiographic sacroiliitis according to modified New York criteria was present in 2 cases. Inflammatory spine and SIJ lesions were detected by MRI in 1 patient. Extra-articular involvement including enthesitis (1 patient), interstitial lung disease (1 patient) and scleritis (1 patient) was also noted. The clinical and demographic characteristics of the OA are given in Table 1.ConclusionThere was a high prevalence of inflammatory arthritis (2 axSpA; 1 RA) in OA (50%) which contradicts with the common concept that OA is a degenerative disorder. According to our results, inflammatory disease should be carefully screened in OA patients as accumulated metabolic products may trigger inflammatory pathways.References[1] Phornphutkul, C., et al., Natural history of alkaptonuria. New England journal of medicine, 2002. 347(26): p.2111-2121.Disclosure of InterestsNone declared
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