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    Alagille Syndrome: A review
    (2013) Callea M.; Bahsi E.; Montanari M.; Ince B.; Mancini G.E.; Yavuz Y.; Radovich F.
    Alagille Syndrome (AGS) is a genetically determined multisystem disorder affecting liver, hearth, eyes, skeleton and facies, less commonly kidney and CNS. The prognosis depends on the severity of the associated anomalies. The liver pathology plays a central role in that most clinical complications are due to long standing cholestasis as a consequence of lack of bile excretion secondary to paucity/absence of interlobular bile ducts. That results in hyperbilirubinemia, hypercholesterolemia, hypertriglyceridemia, fat and liposoluble vitamin malabsorption, pruritus and cutaneous xanthoma. Liver transplantation represents the only curative therapy for the liver pathology. Most hepatic symptoms reverse after liver transplantation. Therapeutical education for oro-dental hygiene is required before and after liver transplantation. The green pigmentation of teeth requires dental rehabilitation. This paper reviews the clinical manifestations of AGS with special regard to the cephalic district, and highlights the necessity for a multidisciplinary approach in order to minimize complications and to ameliorate the quality of life in AGS patients.
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    Bifid uvula and submucous cleft palate in cornelia de lange syndrome
    (2011) Callea M.; Montanari M.; Radovich F.; Clarich G.; Yavuz I.
    Cornelia de Lange syndrome is a rare congenital disease characterized by growth and psychomotor retardation, peculiar facial feature as skeletal and craniofacial deformities, gastrointestinal and cardiac problems and malformation of the upper limb. The prevalence is estimated around 0.6/100000 in the population1. The diagnosis is based on clinical findings and the etiology is still unclear. We present a case of a 17-year-old patient, who came to our attention for dental pain. After an oral examination carried out under general anesthesia the patients presented most of the charactericts described in the literature as micrognathia, high arched palte, delayed aruption, missing of some teeth. The most peculiar findings were the bifid uvula and the submucous cleft palate. The entity of clefting can be determined only with a Magnetic Resonance Imaging which should be carried out under general anesthesia. Caries and periodontal disease were present and the entire dental treatment has been carried out in one sitting without any anestesiologic problems2-5.
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    Dental phenotype in a patient with hypoidrotic ectodermal dysplasia and severe immunodeficiency
    (2011) Callea M.; Faletra F.; Maestro A.; Verzegnassi F.; Rabusin M.; Vinciguerra A.; Radovich F.
    Ectodermal dysplasia is a rare disease which affects at least two ectoderm-derived structures such as hair, nails, skin, sweat glands and teeth. The dentition is altered in number and shape. A 14-year-old male patient with hypodontia, micrognathia, ankylosed teeth and conical shaped teeth was referred for examination, evaluation and treatment. The child exhibited the classic dental phenotype of Ectodermal Dysplasia plus a severe immunodeficiency. Radiographic examination revealed ankylosed primary molars. Ocular findings are reported. Conservative dentistry to reduce the abnormal shape was carried out, and an ultrasound scaling every 4 months, with a strong follow up established. The child fulfilled a good occlusion. Every 3 months the patient has been seen in our department for control of hard and soft tissue in the mouth and after 36 months the dental situation is very well accomplished by patient, family and dental staff. Oral rehabilitation must be carried out at the earliest age possible in order to maintain and correct the oral functions, alignment, good occlusion and a good compliance in smiling and feeding.