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AIM: To estimate the burden of undiagnosed celiac disease (CD) in the Mediterranean area in terms of morbidity, mortality and health cost. METHODS: For statistics regarding the population of each country in the Mediterranean area, we accessed authoritative international sources (World Bank, World Health Organization and United Nations). The prevalence of CD was obtained for most countries from published reports. An overall prevalence rate of 1% cases/total population was finally estimated to represent the frequency of the disease in the area, since none of the available confidence intervals of the reported rates significantly excluded this rate. The distribution of symptoms and complications was obtained from reliable reports in the same cohort. A standardized mortality rate of 1.8 was obtained from recent reports. Crude health cost was estimated for the years between symptoms and diagnosis for adults and children, and was standardized for purchasing power parity to account for the different economic profiles amongst Mediterranean countries. RESULTS: In the next 10 years, the Mediterranean area will have about half a billion inhabitants, of which 120 million will be children. The projected number of CD diagnoses in 2020 is 5 million cases (1 million celiac children), with a relative increase of 11% compared to 2010. Based on the 2010 rate, there will be about 550 000 symptomatic adults and about 240 000 sick children: 85% of the symptomatic patients will suffer from gastrointestinal complaints, 40% are likely to have anemia, 30% will likely have osteopenia, 20% of children will have short stature, and 10% will have abnormal liver enzymes. The estimated standardized medical costs for symptomatic celiac patients during the delay between symptom onset and diagnosis (mean 6 years for adults, 2 years for children) will be about €4 billion (€387 million for children) over the next 10 years. A delay in diagnosis is expected to increase mortal ity: about 600 000 celiac patients will die in the next 10 years, with an excess of 44.4% vs age- and sex-matched controls. CONCLUSION: In the near future, the burden of CD will increase tremendously. Few Mediterranean countries are able to face this expanding epidemic alone., This project was supported by Italian Ministry of Health, Direction of International Affairs, Project MEDICEL., peer-reviewed

AIM: To estimate the burden of undiagnosed celiac disease (CD) in the Mediterranean area in terms of morbidity, mortality and health cost. METHODS: For statistics regarding the population of each country in the Mediterranean area, we accessed authoritative international sources (World Bank, World Health Organization and United Nations). The prevalence of CD was obtained for most countries from published reports. An overall prevalence rate of 1% cases/total population was finally estimated to represent the frequency of the disease in the area, since none of the available confidence intervals of the reported rates significantly excluded this rate. The distribution of symptoms and complications was obtained from reliable reports in the same cohort. A standardized mortality rate of 1.8 was obtained from recent reports. Crude health cost was estimated for the years between symptoms and diagnosis for adults and children, and was standardized for purchasing power parity to account for the different economic profiles amongst Mediterranean countries. RESULTS: In the next 10 years, the Mediterranean area will have about half a billion inhabitants, of which 120 million will be children. The projected number of CD diagnoses in 2020 is 5 million cases (1 million celiac children), with a relative increase of 11% compared to 2010. Based on the 2010 rate, there will be about 550 000 symptomatic adults and about 240 000 sick children: 85% of the symptomatic patients will suffer from gastrointestinal complaints, 40% are likely to have anemia, 30% will likely have osteopenia, 20% of children will have short stature, and 10% will have abnormal liver enzymes. The estimated standardized medical costs for symptomatic celiac patients during the delay between symptom onset and diagnosis (mean 6 years for adults, 2 years for children) will be about €4 billion (€387 million for children) over the next 10 years. A delay in diagnosis is expected to increase mortal ity: about 600 000 celiac patients will die in the next 10 years, with an excess of 44.4% vs age- and sex-matched controls. CONCLUSION: In the near future, the burden of CD will increase tremendously. Few Mediterranean countries are able to face this expanding epidemic alone. © 2011 Baishideng. All rights reserved.

Background & Aims: Celiac disease (CD) is characterized by an inflammatory response to wheat gluten, rye, and barley proteins. Fermentation of wheat flour with sourdough lactobacilli and fungal proteases decreases the concentration of gluten. We evaluated the safety of daily administration of baked goods made from this hydrolyzed form of wheat flour to patients with CD. Methods: Patients were randomly assigned to consumption of 200 g per day of natural flour baked goods (NFBG) (80,127 ppm gluten; n = 6), extensively hydrolyzed flour baked goods (S1BG) (2480 ppm residual gluten; n = 2), or fully hydrolyzed baked goods (S2BG) (8 ppm residual gluten; n = 5) for 60 days. Results: Two of the 6 patients who consumed NFBG discontinued the challenge because of symptoms; all had increased levels of anti-tissue transglutaminase (tTG) antibodies and small bowel deterioration. The 2 patients who ate the S1BG goods had no clinical complaints but developed subtotal atrophy. The 5 patients who ate the S2BG had no clinical complaints; their levels of anti-tTG antibodies did not increase, and their Marsh grades of small intestinal mucosa did not change. Conclusions: A 60-day diet of baked goods made from hydrolyzed wheat flour, manufactured with sourdough lactobacilli and fungal proteases, was not toxic to patients with CD. A combined analysis of serologic, morphometric, and immunohistochemical parameters is the most accurate method to assess new therapies for this disorder.

Background Juvenile dermatomyositis (JDM) is the most frequent idiopathic inflammatory myopathy, characterized by vasculopathy of the skin and/or muscles causing symmetrical proximal weakness and typical skin rashes. In 1975 Bohan and Peter published a classification for dermatomyositis: a certain diagnosis can be obtained with the presence of typical exanthema and at least 3 of the following signs or symptoms: proximal symmetric muscle weakness, elevation of creatine kinase (CK), muscle biopsy and electroneuromyography. Objectives To suggest it is useful to follow less specific muscle enzymes, such as aldolase, in JDM and normal CPK levels. Methods We reviewed 22 patients (8 males and 14 females, median age 8,2 years), with the diagnosis of JDG based on Bohan and Peter criteria. Skin signs preceded muscle manifestations in 7 patients (33%). The diseases was monophasic in 12 (57%) and polyphasic in 9 (43%) patients; one patient was lost at follow up; one patient died of cerebral hemorragia and another patient died of macrophagic activation syndrome. In all patient muscle enzymes were dosed: CK, aspartate amynotraspherase (AST), lactic dehydrogenase (LDH) and aldolase. Results 5/22 (23%, 2 males and 3 females) patients had normal CK: at the beginning 2 of them presented muscle sympthoms (weakness, myalgia, walking difficulty) and cutaneous signs; 3 presented only cutaneous signs. In 3 of 4 patients was observed isolated alteration of aldolase and one of them was affected by a severe hepatic disease with cholestasis (elevated gamma glutamyl transferase). In 1 of them JDM was diagnosed on the basis of cutaneous and muscle signs and symptoms, elevated aldolase ad muscle alteration on magnetic resonance (negative muscle biopsy and electroneuromyography). From literature we know that in 5-10% cases of JDM can be found normal levels of CK. In our cohort 20% of patients with early muscle symptoms has normal CK levels and in 10% normal muscle enzymes. Conclusions The use of aldolase compared with CK in muscle disorders is debated: aldolase can be altered in different diseases, such as erythrocyte diseases or hepatic disorders. CK can be negative because of the presence of inhibitors released by damaged muscle cells, muscle atrophy in advanced stages, concomitant hepatopathy or connective tissue diseases, steroid therapy or elevated acid uric and cystine levels that interfere with enzyme dosage. In suspicious cases of JDM it is useful to combine different enzymes to increase the diagnostic sensibility of this tests, associated with instrumental investigations if one them results negative. References Carter JD, Kanik KS, Vasey FB. Dermatomyositis with Normal Creatine Kinase and Elevated Aldolase Levels. J Rheumatol 2001;28;2366-2367 Gowdie PJ, Allen RC, Kornberg AJ, Akikusa JD. Clinical features and disease course of patients with juvenile dermatomyositis. Int J Rheum Dis. 2013 Oct;16(5):561-7. doi: 10.1111/1756-185X.12107. Epub 2013 Jun 3. Disclosure of Interest None declared

Background PLE has been seldom associated to SLE. Even more rarely, PLE has been described in infancy, as initial and unique clinical presentation of SLE. Methods Clinical case: VM an adopted 10-year-old Vietnamese child, was first admitted to a hospital for sudden generalized oedema at 8 years of age. On admission, hypoalbuminaemia, hypocomplementaemia and a 24-h urinary protein quantification of 610 mg were noticed. For a nephrotic syndrome suspicion, he received daily oral prednisone for 1 month, followed by 4 months of steroid tapering, with complete recovery. One year later, he was again admitted to a hospital for sudden generalized oedema. Laboratory studies showed hypoalbuminaemia, hypocomplementaemia and a 24-h urinary protein quantification of 346 mg. ANAs and dsDNA antibodies were highly positive; serum IgG were elevated too. Because of the suspicion of a nephrotic syndrome relapse, he received a new course of daily oral prednisone for 3 months, followed again by a complete normalization of albumin blood values. After a prednisone free period of 9 months, the patient was admitted to our unit for a third episode of sudden generalized oedema. At clinical examination a conspicuous limbs and periorbital oedema was present. The blood albumin was so low to be undetectable. Again, liver and kidney function were normal, such as 24-h urinary protein. ANAs were positive with speckled appearance, while dsDNA antibodies were absent. Complement was normal. Fecal alpha-1 antitrypsin was of 7.21 mg/g (normal range 0.38-0.5). The Tc-labelled human serum albumin scintigraphy confirmed a severe PLE, mostly deriving from the duodenojejunal area. Gastrointestinal endoscopies showed gastric hypertrophic folds with mucosal nodularity, and lymphoid nodular hyperplasia to the distal ileum. Biopsies revealed a gastric mucosal chronic inflammation with foveolar hyperplasia and microscopic lymphangiectasias. The patient was first started to a nutritional replacement using high-protein diet and medium-chain triglycerides, without a significant improvement of serum albumin. The diagnosis of LUPLE (LUpus Protein Losing Enteropathy) was suspected. Treatment with alternate days i.v. methylprednisolone (20 mg/kg/day) was commenced, followed after one week by daily oral prednisone (1 mg/kg/day) combined to daily oral azathioprine (2 mg/kg/day), with a progressive improvement in albumin blood values. Conclusions PLE is a rare manifestation of autoimmune diseases, including SLE, but the underlying autoimmune disorder is not necessarily apparent at the time of onset of PLE. This case highlights the need of considering PLE as possible etiology of acute hypoalbuminaemia in apparently otherwise healthy children, even without any gastrointestinal symptom. In addition, it is a rare example of PLE as initial and unique clinical presentation of an autoimmune process in childhood. References Al-Mogairen SM. Lupus protein-losing enteropathy (LUPLE): a systematic review. Rheumatol Int 2011;31:995-1001. Awazawa R, Yamamoto Y, Mine Y et al. Systemic lupus erythematosus complicated with protein-losing enteropathy: a case report and review of the published works. J Dermatol 2012;39:454-61. Disclosure of Interest None declared