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UN BAMBINO DI 4 ANNI, AFFETTO DA SINDROME DEL NEVO EPIDERMOLITICO, RITARDO MENTALE E CARDIOPATIA CONGENITA CIANOGENA, SI È PRESENTATO ALLA NOSTRA ATTENZIONE PER UNA VALUTAZIONE ODONTOSTOMATOLOGICA. L’ESAME OBIETTIVO HA EVIDENZIATO EMIPERTROFIA DESTRA DELLA FACCIA E DELLA LINGUA, FORMULA DENTARIA DECIDUA COMPLETA E NELLA SEDE DELL’EMIPERTROFIA, PRESENZA DELL’ELEMENTO 1.6 E 4.6, IGIENE ORALE INSUFFICIENTE E LESIONI CARIOSE DESTRUENTI CON PRESENZA DI PATOLOGIE PERIAPICALI SUPPURATIVE. I GENITORI HANNO RIFERITO LA NECESSITÀ DI RIPETUTE TERAPIE ANTIBIOTICHE PER ASCESSI RICORRENTI. IN RELAZIONE ALLA NECESSITÀ DI UNA RISOLUZIONE TERAPEUTICA IN TEMPI RIDOTTI, AL FINE DI ELIMINARE E LE INFEZIONI E DI DIMINUIRE IL RISCHIO DI ENDOCARDITE BATTERICA, A CAUSA DELLA MANACNZA DI COLLABORAZIONE, SI È DECISO DI TRATTARE IL PAZIENTE IN ANESTESIA GENERALE. NELLA SEDUTA OPERATORIA (DICEMBRE 2001) SONO STATE EFFETTUATE LE TERAPIE CONSERVATIVE DEGLI ELEMENTI DECIDUI RECUPERABILI E DEI PERMANENTI, L’ESTRAZIONE DEGLI ELEMENTI DECIDUI AFFETTI DA CARIE DESTRUENTI E IGIENE ORALE PROFESSIONALE. IN SEGUITO IL BAMBINO È STATO INSERITO IN UN PROGRAMMA DI CONTROLLI IGIENICI BIMESTRALI NEL CORSO DEI QUALI SI È RISCONTRATO UN GRADUALE E PROGRESSIVO AUMENTO DELLA COLLABORAZIONE. LA TEMPESTIVA RISOLUZIONE DELLA SINTOMATOLOGIA DOLOROSA HA PERMESSO UNA GRADUALITÀ NELL’APPROCCIO CHE HA CONSENTITO DI GUADAGNARE E MANTENERE LA COMPLIANCE ALLA SEDUTA ODONTOIATRICA ANCHE IN UN BAMBINO STRESSATO DALLE NUMEROSE OSPEDALIZZAZIONI E CON RITARDO MENTALE.

The clinical case of a boy aged 6 years suffering from Alagille syndrome is described. This is a rare genetic disease characterised, from the hepatic point of view, by anomalies of the intrahepatic biliary ducts (in terms of number, histology and immunohistochemistry) with cholestasis; from the systemic point of view it is characterised by anomalies of various systems (heart, musculoskeletal system, eyes, face, kidneys and nervous system). The patient was referred for dental consultation by the Pediatric Cardiology Section, Sant'Orsola-Malpighi Polyclinic, Bologna City Hospital. Medical history indicated liver transplantation at 3 years; immunosuppressive treatment; heart disease with low risk of bacterial endocarditis (interventricular defect and systolic murmur). Objective examination revealed a dental formula compatible with his age, of a decided green colour (above all the permanent dentition) with numerous caries lesions on the posterior deciduous teeth. In agreeing the treatment plan with the cardiologist (treatment of caries and sealing the fissures and cracks in the permanent first molars) it was decided that antibiotic prophylaxis was not required since such conservative treatment is not at risk for bacterial endocarditis. The patient returned 15 min after the end of the second session with marked swelling of the right upper hemi-lip. Having excluded traumatic origin, a hypothesis was formulated of hypersensitivity to products used during the dental session, in particular talcum-coated gloves and latex rubber dam. The patient was referred immediately to the Dermatology Service, Sant'Orsola-Malpighi Polyclinic, for the appropriate dermatological tests (prick test and epicutaneous test) which confirmed the suspected diagnosis: urticaria from contact with latex materials. The clinical case described confirms the need for the dentist of an interdisciplinary approach in patients suffering from systemic diseases and/or syndromes.

Cornelia de Lange Syndrome (CdLS) is a very rare syndrome characterised by multiple congenital anomaly affecting various organs and severe mental retardation. Incidence has been reported to be 1: 10.000-20.000 among the general population, with no racial predilection. The aetiology is still unknown but researchers, in 2004, discovered a mutation of the NIPBL gene located on chromosome 5 which is considered to be responsible of the disease. The main clinical features of the syndrome regard distinctive facial features, severe growth retardation, developmental and mental delay, hirsutism, structural limb abnormalities. The authors describe the main features of the syndrome focusing on oral and facial malformations and report a case of a three years old patient with CdLS.

The authors describe dental and periodontal conditions of two Chinese sisters affected by familial hypomagnesemia with hypercalciuria and nefrocalcinosis (FHHNC). FHHNC is a rare syndrome, genetically investigated since 1999, transmitted as an autosomal recessive disease. It is related to a mutation of PCN-1 gene which encodes for a tight junction protein named paracellin. CASE REPORT: Oral examination of two Chinese patients showed marked enamel hypoplasia, acute gingivitis and periodontal bone loss and severe malocclusion. The literature does not report previous investigations about the dental conditions of FHHNC patients. The syndrome is not definitely described from the clinical point of view. CONCLUSION: Further researches are necessary to understand the linkage between bone loss and enamel structure anomalies in FHHNC and to discover the relationships between nefrocalcinosis, hypomagnesemia, hypercalciuria and amelogenesis imperfecta. Background The authors describe dental and periodontal conditions of two Chinese sisters affected by familial hypomagnesemia with hypercalciuria and nefrocalcinosis (FHHNC). FHHNC is a rare syndrome, genetically investigated since 1999, transmitted as an autosomal recessive disease. It is related to a mutation of PCN-1 gene which encodes for a tight junction protein named paracellin. Case report Oral examination of two Chinese patients showed marked enamel hypoplasia, acute gingivitis and periodontal bone loss and severe malocclusion. The literature does not report previous investigations about the dental conditions of FHHNC patients. The syndrome is not definitely described from the clinical point of view. Conclusion Further researches are necessary to understand the linkage between bone loss and enamel structure anomalies in FHHNC and to discover the relationships between nefrocalcinosis, hypomagnesemia, hypercalciuria and amelogenesis imperfecta.

This was to investigate the dental characteristics (caries sensitivity and tooth crown size of permanent elements) in subjects affected by Turner Syndrome (TS). PATIENTS AND METHODS: A group of 25 patients affected by TS, aged from 4 to 18 years, was selected and the data were compared to those of an age matched control healthy group. The caries index values in TS patients are higher in the permanent (6.4 vs. 3.9), mixed (0.5 vs. 0.75) and primary dentition (0 vs. 1). The mesio-distal diameter in TS patients was significantly reduced for every tooth measured (in particular for the lower first permanent molar). RESULTS AND CONCLUSION: This investigation confirms that numeric aberration of the X chromosome most likely affects the quantitative and qualitative excretion of amelogenin so that teeth often present enamel defects (reduced crown size and enamel hypoplasia). High caries index values (DMFT) highlight the demand of early preventive measures mostly focused on special care patients.

un caso di riabilitazione ortopedica del mascellare superiore in un paziente affetto da displasia cleidocranica

The rapid improvement in oral health conditions in childhood and adolescence and the ceaseless bombardment of information from the mass media have, during little more than 30 years, brought about a decrease in the prevalence of caries of more than 70% in most European Union countries. The study aimed to take an accurate picture of oral health conditions and dental prevention habits among adolescents. METHODS: The research was conducted on a group of 340 adolescents (12-16 years) in the province of Bologna (Imola) and used the SPSS to compare clinical and epidemiological parameters with medical-history data traditionally understood to be determinant for caries. RESULTS: From an analysis of the data it emerged that the mean value of the decay, missing or filled tooth (DMFT) index in the group was 1.8765 +/- 2.1987 and that the plaque index (according to Löe and Silness) was 1.2519 +/- 0.4213. CONCLUSIONS: Crossing the data obtained, a highly significant correlation emerged (P=0.004) between plaque and caries indexes, and confirmed the primary role of bacterial plaque in causing caries. Unlike what we expected and unlike data reported in the international literature, the caries index was not found to be correlated in a statistically significant way with frequency of brushing, ''preferential consumption of sweet snacks'' (P=0.231) nor ''eating sweets and chewing gum'' (P=0.159) or with taking fluoride tablets (P=0.406). The key to success during adolescence is rooted in childhood; it is the pediatricians' and parents' job to guide and control the acquisition of correct dietary and oral hygiene habits.