Increased percentages of tumor necrosis factor-alpha+/interferon-gamma+ T [corrected] lymphocytes and calprotectin+/tumor necrosis factor-alpha+ monocytes in patients with acute Kawasaki disease.

In vivo exposure to microorganisms resident in the oral cavity is considered as a possible cause of Kawasaki disease (KD), and some epitopes derived from streptococci display homology with Factor H of Complement. Additionally, calprotectin, a major calcium binding protein released by neutrophils and activated monocytes, could be directly involved in endothelial damage occurring in KD. The aim of our study is to evaluate the percentages of IFN-gamma+ and/or TNF-alpha+ lymphocytes and double positive calprotectin/TNF-alpha monocytes (CD14+) after in vitro stimulation with streptococcal- and/or Factor H-derived peptides, in patients with acute KD. Peripheral Blood Mononuclear Cells (PBMCs) obtained from KD patients and febrile controls were stimulated in vitro with peptides. After culture, cells were collected, stained with fluorochrome-labelled monoclonal antibodies against CD3, CD14, calprotectin, IFN-gamma and TNF-alpha, and cytofluorimetric analyses were performed. Our results showed increased percentages of TNF-alpha+/IFN-gamma+ lymphocytes in KD patients in respect to controls when PBMCs were stimulated with streptococcal or Factor H-derived epitopes. In addition, also calprotectin+/TNF-alpha+ monocytes from KD patients were activated after PBMC in vitro stimulation. These findings lead us to speculate that some peptides, derived from oral streptococci and cross-reactive with the human Factor H of Complement, could induce lymphocyte and monocyte activation potentially involved in the pathogenesis of KD. Our results should be confirmed by further studies enrolling more patients and controls than those analyzed in our study.

Internationally adopted children: a new challenge for pediatricians.

Children adopted from abroad by Italian families have increased during the last years. Since 2001 to 2004 they have been more than 10,000, mainly from Eastern Europe, and all indications suggest that they will continue to increase. Most of the internationally adopted children reside in orphanage before adoption where they may experience malnutrition, exposure to infectious diseases, environmental deprivation, neglect. Moreover, their pre-adoptive records are scarcely reliable and their immunization status is not always adequate. The most common long-term problems of internationally adopted children concern developmental and scholastic delay especially if they come from a long and severely deprived institutional setting, precocious puberty and, during adolescence, depressive disorders as well as antisocial behaviours. Inter-country adopted children are at increased risk for health and social problems and have to be recognized as a group of subjects requiring special medical attentions. Specialized centres for internationally adopted children where they could receive medical evaluations upon arrival and a prolonged health follow-up should be set up.

TNFalpha, IFNgamma and IL-10 gene polymorphisms in a sample of Sicilian patients with coeliac disease.

BACKGROUND: Coeliac disease is associated with DQ2 and DQ8 alleles, but other genes also confer an additional genetic risk. AIMS: Defining whether the genetic profiles of interleukin-10, tumour necrosis factor alpha and interferon gamma are associated with an increased coeliac disease risk. PATIENTS AND METHODS: The functionally gene polymorphisms of tumour necrosis factor alpha (-308G/A), interferon gamma (+874T/A) and interleukin-10 (-1082G/A) were typed using sequence specific primer-polymerase chain reaction in 110 Sicilian coeliac disease patients and in 220 Sicilian healthy controls. RESULTS: No differences in genotype frequencies of interleukin-10 polymorphisms were found between coeliac disease patients and healthy controls. A significant increase of -308A (p<0.033; OR: 1.72; CI: 1.27-2.33) and of +874T (p: 0.0045; OR: 3.02; CI: 1.47-6.21) allele frequencies, both in hetero- and homozygosis, was observed in coeliac patients in comparison with healthy controls. In addition, simultaneous significant higher percentages of -308A and +874T alleles (p: 0.0066; OR: 2.33; CI: 1.42-3.82) as well as simultaneous significant lower percentages of -308A and +874T alleles (p: 0.003; OR: 0.23; CI: 0.10-0.60) were observed in coeliac patients compared with healthy controls. CONCLUSIONS: Genetically determined higher frequencies of -308A tumour necrosis factor alpha and +874T interferon gamma alleles, both in hetero and in homozygosis and mostly whether simultaneous, may play a role in predisposing to gluten intolerance. Subjects positive for -308A tumour necrosis factor alpha and +874T interferon gamma alleles have an increased risk for coeliac disease.

An interesting question of Pompe disease. A case report.

Glycogenosis type II or Pompe disease is an inherited autosomal recessive disorder known in 3 different clinical forms (infantile, juvenile and adult). We report on a case diagnosed as a classic infantile form with the worst outcome of all 3 described, if we had followed and executed a correct and complete diagnostic pathway. A 7 months old female child was admitted for fever and dyspnoea. At chest auscultation weepings and weezings were found; on the cardiac apex a murmur due to mitralic failure was retrieved. The thorax X-ray showed a greatly increased heart shadow with a cardiothoracic index of 0.75. ECG showed high voltages and signs of bilateral ventricular hypertrophy. Cardiac ultrasonography confirmed the presence of a big heart with an enormous swollen left ventricle and a severe mitralic failure. The clinical diagnosis of infantile Pompe disease was confirmed by the almost total absence of cellular acid a-glucosidase activity but we couldn't perform the assay because of the rapid exitus of our patient, which occurred before glycogen storage disease II was suspected. So, we tried to compare our case with others reported in the literature in order to ratify our diagnostic hypothesis. The contribution of genetic counseling practiced on all the couples at risk remains useful every time that a certain diagnosis is made.

Epidemiological and clinical features in immigrant children with coeliac disease: an Italian multicentre study.

BACKGROUND: There are no available data concerning the incidence and the clinical pattern of coeliac disease in immigrant children coming to Italy from developing countries. AIMS: To evaluate the epidemiological and clinical features of coeliac immigrant children coming to Italy. PATIENTS AND METHODS: Hospital records of 1917 children diagnosed in 22 Italian Centres from 1999 to 2001 as having coeliac disease were retrospectively reviewed, comparing immigrant patients versus Italian ones. RESULTS: 36/1917 (1.9%) coeliac children were immigrant. This prevalence was similar to that of the immigrant children among the whole paediatric population living in Italy. Prevalence was influenced by geographical factors, being higher in Northern Italy (1.7%) and in Central Italy (2.5%) than in Southern-Insular Italy (1.5%), as consequence of a higher proportion of immigrants in these regions. The native areas of the immigrant children were East Europe (15/36), Northern Africa (14/36), Southern Asia (4/36), West Africa (1/36), East Africa (1/36) and the Middle East (1/36). The clinical spectrum and dietary habits in immigrant patients were similar to those of the Italian children. CONCLUSIONS: Coeliac disease among the immigrant children coming from developing countries is an emerging problem, and physicians need to be fully aware of it. An important risk factor for coeliac disease in immigrant children appears to be sharing of the same dietary habits with the Italian population. The finding of coeliac disease in children coming from many countries worldwide suggests that coeliac disease is a global public health problem.

The global village of celiac disease.

In the last years our knowledge on epidemiology of celiac disease has increased: there is a wide spectrum of its clinical presentation (classical, atypical, silent and latent forms of celiac disease), and of its pathological mucosal intestinal features, which range from early and mild pictures to severe villous atrophy (Marsh stages). In addition, a strong genetic component, associated with the susceptibility to the disease (HLA and non HLA genes), has been found. This knowledge, together with the availability of new high sensitive and specific serological tests (antigliadin, antiendomysium and antitransglutaminase antibodies), has led us to the realization that celiac disease is the most common food intolerance in the world, involving genetically predisposed individuals consuming gluten-containing cereals in their diet. So, today it is well known that celiac disease is a common disorder not only in Europe but also in populations of European ancestry (North and South Americas, Australia), in North Africa, in the Middle East and in South Asia, where until a few years ago it was historically considered extremely rare. Therefore, celiac disease is spread worldwide as in a common "Global Village", and greater levels of awareness and attention on gluten intolerance are needed, both in the Old and in the New World.

Mycoplasma pneumonias distribution, epidemiology and prevalence in a triennial survey.

OBJECTIVES: To evaluate: (1) the incidence of pneumonia monthly distribution; (2) the rate of pneumonitis due to Mycoplasma Pneumoniae; (3) the suitability of choosing an empirical-based antibiotic-therapy; (4) the need of a critical revision of Mycoplasma serological data. PATIENTS AND METHODS: We studied 188 patients admitted to the Palermo University Pediatrics Department, from september 1998 to august 2001, with admission diagnosis of pneumonia. RESULTS: The highest incidence of pneumonia was in december and march as in both months 28 cases occurred in the whole period 1998-2001 (average of 9.3 cases per each month). The highest rate of pneumonias by Mycoplasma Pneumoniae was in may with a total number of 11/21 cases (52.3%, average of 3.67 cases per month) in the above mentioned three-year period. CONCLUSIONS: Incidence of Mycoplasma pneumonitis is more represented in subjects that are at school age. Our study confirms the enormous variability of the prevalence of the infection by Mycoplasma Pneumoniae and how difficult it is to make an accurate diagnosis lacking standardized, rapid, specific and comparative methods.

Two strange cases of hypereosinophilia and child's relapsing angio-oedema.

Two patients aged two and four years came to our observation with "angio-oedema" and relapsing hypodermitis. Atopic diseases were present in both family histories. The clinical examination of both children revealed a good nutritional status, the presence of angio-oedema with fleeting (48 hours max length) and localized hypodermic infiltrations. Amongst laboratory investigations, the blood cells count and the total count of Immunoglobulins showed hypereosinophilia and increased IgE levels over ten times the normal values. Prick tests for the most common inhalant and nutritive allergens were negative. A strong positivity of ELISA for Taenia Solium metacestodes on both sera samples suggested a diagnosis of human cysticercosis.