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      Oral Facial Manifestations of Sanjad–Sakati Syndrome: A Literature Review

      Children
      MDPI AG

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          Abstract

          Aim: To perform a comprehensive review of orofacial manifestations of Sanjad–Sakati syndrome (SSS). Methods: A comprehensive electronic literature search was performed using PubMed, Scopus and Cochrane library databases. The search keywords included were “Sanjad–Sakati syndrome (SSS)”, “dental manifestations”, “dental management”, “oral health”, “dental care for patients with SSS”, “dental health of people with SSS”, “caries”, and “oral hygiene”. The inclusion criteria were papers published only in English, papers published by August 2021, and papers discussing orofacial manifestations of SSS and language. Results: The search of the databases retrieved eleven case reports and three case series studies. Overall, 56 cases (11 case reports and 3 case series studies) were reported on Sanjad–Sakati syndrome in the published literature. The majority of the reports are from the Middle Eastern region. Conclusions: The reported orofacial manifestations of SSS include beaked nose, depressed nasal bridge, enamel hypoplasia, hypodontia, low-set ears, posteriorly rotated ears, deep-set eyes, microcephaly, microdontia, micrognathia, prominent forehead, retrognathia, and thin lips. The review paper also establishes the importance of the dental under general anesthesia in SSS individuals.

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          Most cited references60

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          Clinical practice. Hypoparathyroidism.

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            A new syndrome of congenital hypoparathyroidism, severe growth failure, and dysmorphic features.

            Twelve infants (six boys, six girls) with severe hypocalcaemic tetany or convulsions were seen over a three year period. Nine patients were symptomatic in the newborn period. Their hypocalcaemia was associated with hyperphosphataemia and very low concentrations of immunoreactive parathyroid hormone. None of the babies suffered from congenital cardiac disease. Cell mediated immunity, measured in five patients, was normal. There were no chromosomal abnormalities but all patients shared several dysmorphic features including deep set eyes, microcephaly, thin lips, beaked nose tip, external ear anomalies, micrognathia, and depressed nasal bridge. Mental retardation of varying degree was found in all patients. All had severe intrauterine and postnatal growth retardation. Four patients have died. The remaining eight patients are on treatments with vitamin D and calcium supplements with no change in their growth pattern. We believe that this association of congenital hypoparathyroidism with severe growth failure and dysmorphism represents a new syndrome.
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              The Multidisciplinary Team (MDT) Approach and Quality of Care

              The core function of a multidisciplinary team (MDT) is to bring together a group of healthcare professionals from different fields in order to determine patients' treatment plan. Most of head and neck cancer (HNC) units are currently led by MDTs that at least include ENT and maxillofacial surgeons, radiation and medical oncologists. HNC often compromise relevant structures of the upper aerodigestive tract involving functions such as speech, swallowing and breathing, among others. The impairment of these functions can significantly impact patients' quality of life and psychosocial status, and highlights the crucial role of specialized nurses, dietitians, psycho-oncologists, social workers, and onco-geriatricians, among others. Hence, these professionals should be integrated in HNC MDTs. In addition, involving translational research teams should also be considered, as it will help reducing the existing gap between basic research and the daily clinical practice. The aim of this comprehensive review is to assess the role of the different supportive disciplines integrated in an MDT and how they help providing a better care to HNC patients during diagnosis, treatment and follow up.
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                Author and article information

                Journal
                Children
                Children
                MDPI AG
                2227-9067
                April 2022
                March 22 2022
                : 9
                : 4
                : 448
                Article
                10.3390/children9040448
                179f9ba9-dd24-4f65-827b-d1d42f0cf75b
                © 2022

                https://creativecommons.org/licenses/by/4.0/

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                Self URI (article page): https://www.mdpi.com/2227-9067/9/4/448

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