Orthodontic treatment in a young patient with Beckwith-Wiedeman Syndrome (BWS) is reported and a multidisciplinary approach to the management of this syndrome is reviewed. The patient presented with a tendency to Class III malocclusion, an open-bite and a slight macroglossia, which was treated at an early age by glossotomy. It was decided to monitor growth without treatment and to wait for the best time to begin therapy. It was based only on functional rehabilitation, without any fixed appliances, in which optimum intercuspation of the teeth and the skeletal Class I relationship was achieved and maintained after the retention period of three years. In conclusion the treatment of BWS patients requires a multidisciplinary approach that includes orthodontics, orthopaedics and surgical intervention. It is also necessary to underline the significance of diagnosis at an early age and timely treatment to reduce the development of dento-skeletal alterations.
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http://dx.doi.org/10.17796/jcpd.27.4.6356k0231w213386 | DOI Listing |
Kurume Med J
November 2024
Department of Obstetrics and Gynecology, Kurume University School of Medicine.
This study aimed to explore the clinical characteristics of umbilical cord cysts (UCCs) detected from the second trimester onwards in a single tertiary center over a 22-year period. The subjects consisted of a total of 12 cases of UCCs among 11 pregnant women (10 singleton pregnancies and 1 twin pregnancy), diagnosed at ≥ 14 weeks of gestation, and managed at Kurume University Hospital from 2000 to 2021. The maternal backgrounds, characteristics of the cysts, including their location, numbers, sizes, associated fetal/neonatal findings, and outcomes, were analyzed retrospectively.
View Article and Find Full Text PDFFront Oncol
October 2020
Department of Paediatric Haematology/Oncology Cell and Gene Therapy, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.
Pediatric adrenocortical tumors (ACTs) are very rare endocrine neoplasms in childhood. In this study, we performed a retrospective analysis of children with ACT treated at our institution by examining clinical and genetic disease features, treatment strategies, and outcomes. We retrospectively analyzed a cohort of 13 children treated at the Bambino Gesù Children's Hospital from November 2010 to March 2020.
View Article and Find Full Text PDFClin Invest Med
February 2016
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In a typical morning in the Cancer Genetics Clinic at The Hospital for Sick Children in Toronto, the following array of patients and families might be seen: a family of three children, all harbouring a mutation of the succinyl dehydrogenase C gene inherited from their father who had had extensive surgery several years ago for a secreting paraganglioma; three families with Li-Fraumeni syndrome, each with at least one child harbouring a TP53 gene mutation conferring a lifetime risk of cancer approaching 100% and currently undergoing surveillance for early tumour detection; two children with Li-Fraumeni syndrome undergoing treatment for cancer - one having had three cancer diagnoses before 19 months of age and the other just completing therapy for metastatic adrenocortical carcinoma at age 3; two children with von Hippel-Lindau disease being monitored for persistent pancreatic neuroendocrine tumors and cerebellar hemangioblastomas, respectively; and one child with Beckwith-Wiedeman syndrome and Wilms tumor and another child completing therapy for a pleuropulmonary blastoma (PPB).
View Article and Find Full Text PDFPediatr Blood Cancer
January 2014
Department of Pediatric Oncology, Institut Curie, Paris, France.
Background: Wilms Tumor (WT) can occur in association with tumor predisposition syndromes and/or with clinical malformations. These associations have not been fully characterized at a clinical and molecular genetic level. This study aims to describe clinical malformations, genetic abnormalities, and tumor predisposition syndromes in patients with WT and to propose guidelines regarding indications for clinical and molecular genetic explorations.
View Article and Find Full Text PDFGynecol Obstet Fertil
November 2008
Service de Gynécologie-Obstétrique, Maternité Aline-de-Crépy, Hôpital Bichat-Claude-Bernard, 75018 Paris, France.
Numerous recent studies involve DNA damages associated with poor fertilization rates, early embryo development defect, and poor quality of conceptus following Assisted Reproductive Technologies (ART). The authors denounce a particularly high rate of miscarriages and childhood cancer or dominant genetic mutations such as achondroplasia, Apert syndrome or aberrant gene imprinting such as Angelman and Beckwith Wiedeman syndromes. Gametes DNA defects have numerous origins which are difficult to determine; they are known to involve hypomethylation, oxydative stress and environmental factors.
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