Celiac disease in Turkey Aydan Kansu Zarife Kuloğlu Ankara University School of Medicine Pediatric Gastroenterology, Hepatology and Nutrition MEDICEL Meeting Naples September

Turkey Population: year: Estimated CD (children): TPGHAN (about 100 members) Pediatric Gastroenterology Departments:30 Celiac society  Ankara  İstanbul  İzmir  Diyarbakır

Epidemiology Ertekin V. J Clin Gastroenterol 2005;8:  6-17 y, 1263, ttg IgA Seropositivity.1/115 Biopsy proven CD:1/158 Demirçeken F. Turk J Gastroenterol 2008;19:14-21  2-18 y, 1000, ttg IgA Seropositivity.1/100 Biopsy proven CD:1/111

Epidemiology Dalgıç B and Turkish Celiac Disease Study Group. ESPGHAN 2010, İstanbul  6-17 y, Two-stratified Cluster sampling, 20190, 62 cities ttG IgA, ttg IgG, EMA Ig A, same pathologist

Epidemiology

Prevalance:1/212 (0.47%)

HLA types Tüysüz B. Tissue Antigens 2001;6:540-2  55 CD&50 control HLA DQA1, DQB1 (PCR –SSP) HLA A10501, HLA DQB102 alles in  than control Kuloğlu Z, Turk J Pediatr 2008;50:  75 CD (45 classic form: 6.7±3.8 y&30 atypical 9.3±4.3y)  100 healthy renal Tx donors HLA typing: Serologically (standart lymphotoxicity techniques) Control group: HLA A29, B51, CW5, DR14, Dr16, DQ1  CD: HLA B13, CW7, B8, DR7, DR17, DQ2 was higher than control HLAB35, DR11, DQ7:classical type HLA B8: Atypical type

Clinical Presentation 109 patients, 8.8±4.6 y  60.6% Classical type  37.6% atypical  1.8% silent Sx: Diarrhea (53.2%), FTT, short stature, abdominal pain PE: paleness (40.4%), underweight (34.8%), short stature (31.2%) Lab  IDA (81.6%), zinc def (64.1%), PT  (35.8%), transaminase  (24.7%)  Ig A deficiency (9.1%), ab (+) (8%), BMD  (28/52), EEG abnormality (4/38)  HLA DQ2 and/orDQ8:91% Abdominal distention, IDA, PT , hypoalbuminemia, transaminase  more frequent in classical type than atypical form

Clinical Presentation Altuntaş B. Acta Pediatr Jpn 1998;40:  47 short statured patients (without GIS symptoms)  Bx proven CD:55% Altuntaş B. Acta Pediatr Jpn 1998;40:597-9  9 patients, ALT   Liver Bx: fibrosis, nonspecific reaction  Duodenal Bx: CD

Clinical Presentation 32 CD (16 recent diagnosis&16 GFD) 100 healthy control  BMD, Ca, P, ALP, PTH (baseline&12 mo) Dual energy radiograph bone densitometer, L1-4, g/cm 2 BMD& BMC were lower in CD than control Osteoporosis was common in recent diagnosis 1 year later BMD values in patients with recent diagnosis significantly increased After 1 year osteopenia was resolved

Clinical Presentation 50 with FMF ( questioned, examined, IgA, AGA IgA, AGA IgG, EMA IgA, bx)  1 EMA (+), bx normal 17 with CD (questioned, examined, lab, mutation analysis for MEFV)  MEFV mutation:23.5% No assosication between CD and FMF

Clinical Presentation Sarı S. Dig Dis Sci 2009;54:830-2  101 autoimmune thyroiditis  103 healthy  Bx proven CD:4.9% Dalgıç B. J Child Neurol 2008;21:6-7  70 epilepsy&103 control  Bx proven CD: 1.8% Alehan F. Cephalalgia 2009;28:945-9  73 migraine&147 control  Bx proven CD:- Kalaycı AG. Acta Paediatr 2005;8:  135 IDA &223 control  Biopsy proven CD:4.4%

Clinical Presentation Selimoğlu MA. J Clin Gastroenterol 2007;7:  126 CD  AST  (51.6%), ALT  (35.7%), CK  (39.7)  Myopathy? Polat TB. Dig Liver Dis 2008;  45 CD&30 control  Subclinical systolic dysfunction of the left ventricule

Follow-up Aydoğdu S. Dig Dis Sci 2009;10:  34 CD, followed for at least four years GFD leads to rapid increase in W SDS and H SDS in patients < 5 y Increase in H SDS is highest in patients 5-10 y Age at diagnosis is the major factor for W SDS and H SDS at follow-up  Early diagnosis and strict GFD are essential for long- term growth

Leptin&Ghrelin&Nitric oxide Ertekin V. J Clin Gastroenterol 2006;10:906-9  19 CD&16 control  Serum leptin level is affected in CD, is not related to histopathology, is responsive to GFD Selimoğlu MA. JClin Gastroenterol 2006;3:191-4  36 CD&10 healthy  Ghrelin is increased in CD and is responsive to GFD Ertekin V. J Clin Gastroenterol 2005;39:  41 CD&14 control  NO level is high in CD non adharent to GFD

Conclusion CD is common in Turkey CD is a well known disease among pediatric gastroenterologists Rising awareness is needed among pediatricians and in primary care and also among society Serologic diagnostic tools are needed to be available routinely Wheat is basic nutrient in Turkish cousine, therefore adherance to GFD may be difficult GFD products are not widely accessible Financial support of the state for GFD is not enough