1.Dental and Skeletal Characteristics and Behavioral Aspects of the Patient with Floating-Harbor Syndrome Compared with Twin Sister
Jonghwa LIM ; Gimin KIM ; Jaesik LEE ; Soonhyeun NAM ; Hyunjung KIM
Journal of Korean Academy of Pediatric Dentistry 2022;49(2):234-240
Floating-Harbor syndrome (FHS) is a rare genetic disorder. This report introduced in a patient with FHS. Distinctive facial characteristics, severe skeletal class 3 malocclusion with underdeveloped maxilla and protruded mandible, congenital missing teeth, microdontia and ectopic positions of maxillary teeth were presented in the patient. In his twin sister, mild skeletal class 3 malocclusion with protruded mandible was observed but congenital missing teeth and microdontia were not observed. High-arched palate, narrow V-shaped maxillary arch compared to wide and ovoid mandibular arch and inverse relationship between the maxillary and mandibular intermolar width resulted in posterior crossbite were confirmed by model analysis of the patient. These were not observed in the twins. Behaviorally, poor cooperation during dental treatment because of mental retardation was observed in the patient.
2.A Retrospective Study on the Treatment of Dens Evaginatus for the Last 5 Years
Jonghwa LIM ; Gimin KIM ; Jaesik LEE ; Soonhyeun NAM ; Hyunjung KIM
Journal of Korean Academy of Pediatric Dentistry 2022;49(2):158-169
The purpose of this study was to analyze treatment methods, results, timings and clinical signs and symptoms in failed cases of each treatment method of dens evaginatus on the premolar areas. In this study, 151 patients and 417 teeth were included. Resin restoration and direct pulp capping as preventive treatment and pulp revascularization, apexification and conventional endodontic treatment as endodontic treatment were included for treatment methods. In the preventive resin restoration, successful results were shown in the intact tubercles and also effective when the tubercles were fractured. In the direct pulp capping, resolved clinical symptoms and growth of the roots were shown when there was only pain during chewing without apical lesion. Apical lesion was the most common pretreatment signs and symptoms of the pulp revascularization, apexification and conventional endodontic treatment. In the pulp revascularization, successful results was obtained in most cases. But in some cases, root length or root wall thickness was not increased. Effective results were shown both of the apexification and conventional endodontic treatment. In order to increase success rate of preventive treatment of dens evaginatus, resin restoration was required to be done when tubercle did not occluded or in the presence of intact tubercles. When tubercle was fractured, root development stage and pulp condition should be considered for successful treatment.
3.Vandetanib for the Management of Advanced Medullary Thyroid Cancer: A Real-World Multicenter Experience
Mijin KIM ; Jee Hee YOON ; Jonghwa AHN ; Min Ji JEON ; Hee Kyung KIM ; Dong Jun LIM ; Ho-Cheol KANG ; In Joo KIM ; Young Kee SHONG ; Tae Yong KIM ; Bo Hyun KIM
Endocrinology and Metabolism 2020;35(3):587-594
Background:
Vandetanib is the most widely used tyrosine kinase inhibitor for the treatment of patients with advanced medullary thyroid cancer (MTC). However, only limited data regarding its use outside clinical trials are available. We aimed to evaluate the efficacy and safety of vandetanib in patients with advanced MTC in routine clinical practice.
Methods:
In this multicenter retrospective study, 12 patients with locally advanced or metastatic MTC treated with vandetanib at four tertiary hospitals were included. The primary outcome was the objective response rate (ORR) based on the Response Evaluation Criteria in Solid Tumors. The progression-free survival (PFS), overall survival (OS), and toxicities were also evaluated.
Results:
Eleven patients (92%) had distant metastasis and 10 (83%) had disease progression at enrollment. Partial response was observed in five patients (ORR, 42%) and stable disease lasting ≥24 weeks was reported in an additional five patients (83%). During the median 31.7 months of follow-up, disease progression was seen in five patients (42%); of these, two died due to disease progression. The median PFS was 25.9 months, while the median OS was not reached. All patients experienced adverse events (AEs) which were generally consistent with the known safety profile of vandetanib. Vandetanib was discontinued in two patients due to skin toxicity.
Conclusion
Consistent with the phase III trial, this study confirmed the efficacy of vandetanib for advanced MTC in terms of both ORR and PFS in the real-world setting. Vandetanib was well tolerated in the majority of patients, and there were no fatal AEs.