Yazar "Montanari M." seçeneğine göre listele

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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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    An unusual case: Neurofibromatosis type 5
    (2012) Callea M.; Ulku S.Z.B.; Giustini S.; Yavuz I.; Ulku M.M.; Montanari M.; Basaran G.
    Neurofibromatosis is a disease which includes 5 types in its classification. It has been firstly described by Robert Smith in 1849, but its name comes from the first publication in 1882, in which Von Recklinghausen convinced the scientific and medical world that Neurofibromatosis (NF) was a distinct entity. Cafe-au-lait spots, cutaneous neurofibromas and Lisch nodules are the characteristics of the most common forms. Bilateral acoustic neuromas distinguish the type 2. Type 3, Riccardi type and intestinal type are briefly described. Neurofibromatosis type 5 (NF5) is characterized by cafe-au-lait spots restricted to one area of the body. We report oro-dental phenotype of a patient affected by NF type 5, rare per se and unreported in literature.