RESUMO
Zinsser-Cole-Engmann syndrome also called Dyskeratosis Congenita (DKC) is a rare genodermatosis first described by Zinsser in 1906. Mutations in DKC1 gene is responsible for DKC. It is usually inherited as an X-linked recessive trait, resulting in a striking male predilection. It is characterized by a triad of reticular skin pigmentation, nail dystrophy and mucosal leukoplakia. Complications include predisposition to malignancy and bone marrow failure. Here, we report a case of DKC in a 9-year-old boy with classic triad of signs. Special investigations like endoscopy, barium swallow and bone-marrow aspiration study confirmed the diagnosis. There is no effective treatment for DKC. Some preventive measures can be adopted and the only long term cure for the haematological abnormalities is allogenic haemopoietic stem cell transplantation.
RESUMO
INTRODUCTION: Most children are casual and moderately agreeable in the dental treatment environment, however some of them show practices that upset the professional and make the protected conveyance of worthy treatment extremely troublesome. For such cases dental practitioner utilizes behavior management techniques. At the point when behavioral administration procedures come up short, some type of pharmacologic sedation or anesthesia may be an important and vital option. Dental sedation is a strategy in which the utilization of a medication or drugs produce(s) a condition of depression of the central sensory system empowering treatment to be completed during which verbal contact with the patient is kept up all through the time of sedation. AIM: This study was designed to evaluate and compare the effectiveness of oral midazolam and oral ketamine in combination with N2O-O2 in children undergoing dental treatment. MATERIALS AND METHODS: This study involved a sample of 30 pediatric dental patients (age range is 3-9 years), whose selection criteria included ASA I & II health status, cooperative but apprehensive behavior and a need for multiple dental extractions. The patients were assigned to receive oral midazolam on their first visit and on the follow up visit they received oral ketamine. Nitrous oxide (30%) was used during each sedation visit. Physiological parameters like Respiratory Rate (RR), pulse rate, and oxygen saturation were evaluated for each procedure, followed by the use of modified Bender Visual Motor Gestalt Test to evaluate psychomotor effects. Data were analyzed using Independent sample student t -test. RESULTS: Analysis of the data showed statistically no significant difference (p >0.05) on comparison of effectiveness of oral midazolam-N2O with oral ketamine-N2O when pulse rate, oxygen saturation and respiratory rate were taken into consideration. Psychomotor performance was found to be marginally better with oral midazolam-N2O compared to oral ketamine-N2O. CONCLUSION: Both the drugs were effective in reducing the patient anxiety while undergoing dental extractions. Though the t-test results were not statistically significant with respect to physiological parameters. Oral midazolam-N2O showed marginally better results compared to oral ketamine-N2O with respect to psychomotor effects.
