• The Journal of Korea Assosiation for Disability and Oral Health
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• v.9 no.2
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• pp.107-110
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• 2013

Adrenoleukodystrophy (ALD) is a rare, inherited disorder that leads to progressive brain damage and failure of the adrenal glands. It is passed down from parents to their children as an X-linked genetic trait. Therefore, it affects primarily males especially under the age of 10. People with ALD have excessive accumulation of very long chain fatty acids (VLCFAs) in their brain because they do not have the enzyme which break down these fatty acids. Special method for treatment of ALD is not available. Eating a diet low in VLCFAs and taking special oils, called Lorenzo's oil, can lower the blood levels of VLCFAs. But this oil cannot stop the destruction of nerve cell. This article presents a case report: Dental treatment of a 11-year-old male patient with ALD under general anesthesia. Careful management must be required during treatment procedure because of difficulty in cooperation, seizure disorders, gastroesophageal reflux, pulmonary aspiration and airway complications.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.12 no.2
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• pp.92-95
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• 2016

Beckwith-Wiedemann syndrome comprises multiple congenital anomalies with a risk of development of childhood tumors. Macroglossia is the predominent finding in Beckwith-Wiedemann syndrome and other findings include postnatal somatic gigantism, abdominal wall defects, nevus flammeus of the face, congenital heart defects, and cleft palate. The aim of this case presentation is to describe the dental treatment of a child with Beckwith-Wiedemann syndrome. A 3-year-old boy with Beckwith-Wiedemann syndrome visited to the Seoul National University Dental Hospital for dental evaluation and treatment. Due to the multiple caries and poor cooperation, we planned to perform the dental procedure under general anesthesia. The dental procedure was successfully performed and there was no airway complications after extubation. This case suggests that general anesthesia may be useful for the patient with uncooperative Beckwith-Wiedemann syndrome, since macroglossia and laryngomalacia may exacerbates airway compromise during dental sedation.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.12 no.2
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• pp.72-76
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• 2016

Traumatic dental injury (TDI) is a common problem in children and adolescent. The prevalence of dental trauma among children with disability as compared to healthy children. The TDI of an immature permanent tooth can lead to the loss of pulp vitality and arrested root development. Traditionally, the treatment of choice for necrotic immature tooth is apexification, which is induction of hard tissue barrier at the apex to produce more favorable conditions for conventional root canal filling. This case report describes the treatment of a necrotic immature permanent central incisor with complicated crown fracture. The patient had multiple disabilities (cerebral palsy, congenital heart disease, developmental delay, and gait disturbance) and suffered from She was suffered from repetitive traumatic injury. Apexification and resin restoration was performed under general anesthesia, and favorable clinical results were achieved.

• Journal of Dental Anesthesia and Pain Medicine
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• v.16 no.3
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• pp.213-216
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• 2016

Children with an intellectual disability often demonstrate lack of cooperation during dental treatment and require behavioral management. A child with mild intellectual disability can be managed adequately using restraints and medication. However, in cases of profound intellectual disability, dental treatment under general anesthesia is usually required. In cases where the patient is an intellectually disabled child who has intellectually disabled parents, it is difficult to evaluate the patient's preoperative condition and to obtain consent for treatment under general anesthesia. Furthermore, they are unable to respond to emergencies after treatment. Therefore, dental treatment should be performed under general anesthesia with hospitalization for children with an intellectual disability. This case presents the dental treatment of an intellectually disabled child, who has intellectually disabled parents, and who required general anesthesia and hospitalization.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.25 no.6
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• pp.441-452
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• 2022

Congenital diarrheal disorders (CDDs) with genetic etiology are uncommon hereditary intestinal diseases characterized by chronic, life-threatening, intractable watery diarrhea that starts in infancy. CDDs can be mechanistically divided into osmotic and secretory diarrhea. Congenital tufting enteropathy (CTE), also known as intestinal epithelial dysplasia, is a type of secretory CDD. CTE is a rare autosomal recessive enteropathy that presents with intractable neonatal-onset diarrhea, intestinal failure, severe malnutrition, and parenteral nutrition dependence. Villous atrophy of the intestinal epithelium, crypt hyperplasia, and irregularity of surface enterocytes are the specific pathological findings of CTE. The small intestine and occasionally the colonic mucosa include focal epithelial tufts. In 2008, Sivagnanam et al. discovered that mutations in the epithelial cell adhesion molecule (EpCAM, MIM# 185535) were the genetic cause of CTE (MIM# 613217). More than a hundred mutations have been reported to date. Furthermore, mutations in the serine peptidase inhibitor Kunitz type 2 (SPINT2, MIM# 605124) have been linked to syndromic CTE. In this study, we report the case of a 17-month-old male infant with congenital diarrhea. Despite extensive etiological workup, no etiology could be established before admission to our center. The patient died 15 hours after being admitted to our center in a metabolically decompensated state, probably due to a delay in admission and diagnosis. Molecular autopsy with exome sequencing revealed a previously reported homozygous missense variant, c.757G>A, in EpCAM, which was confirmed by histopathological examination.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.25 no.5
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• pp.406-412
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• 2022

Purpose: The role of endoscopic retrograde cholangiopancreatography (ERCP) in the management of hepatobiliary and pancreatic diseases in the pediatric population was not well defined until recently. Our aim was to determine the feasibility, outcomes, and safety of ERCP in a local pediatric population, particularly using standard adult endoscopes and accessories. Methods: This retrospective study was conducted at the National Hospital of Sri Lanka. Pediatric patients (aged <16 years) who underwent ERCP from January 2015 to December 2020 were included in the study. Data, including patient demographics, indications for the procedure, technical details, and associated complications, were collected from the internal database and patient records maintained at the hospital. Results: The study included 62 patients who underwent a total of 98 ERCP procedures. All the procedures were performed by adult gastroenterologists using standard adult endoscopes and accessories. The mean age was 11.01±3.47 years. Pancreatic diseases were the major indications for most of the procedures (n=81, 82.7%), with chronic pancreatitis being the most common. Seventeen procedures (17.3%) were carried out for biliary diseases. Overall cannulation and technical success rates were 87.8% and 85.7%, respectively. Stent placement was the most common therapeutic intervention (n=66; 67.4%). Post-ERCP pancreatitis was the most common complication, occurring in eight patients (8.2%). Conclusion: ERCP can be successfully and safely performed in pediatric populations using standard adult endoscopes and accessories with complications similar to those of adults. Adult ERCP services can be offered to most pediatric patients without additional costs of pediatric endoscopes and accessories.

• Journal of Dental Anesthesia and Pain Medicine
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• v.23 no.6
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• pp.327-335
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• 2023

Background: Pain control is a crucial aspect of pediatric dentistry for patient management. Thermo-mechanical devices (Buzzy^TM Pain Care Labs, USA) work on the concept of vibration and cooling and have shown promising results in pain control during local anesthesia in pediatric dentistry. On the other hand, audio distraction has also been used for pain management. The amount of pain endured is determined by the patient's perception and attentiveness. Thus, if audio function is added to the thermomechanical device it might increase its efficiency. Hence, the present study aimed to compare pain on injection using a thermo-mechanical device with and without audio during inferior alveolar nerve block (IANB) injection in children aged 5-10 years old. Methods: Twenty-eight children aged between 5 and 10 indicated for IANB were included in this randomized study. Children who were undergoing the dental procedure were divided into 2 groups, with 14 children in each group. The study group was the thermo-mechanical device with audio distraction; the control group was the thermo-mechanical device without audio distraction. IANB was administered. Subjective pain evaluation was performed using the Wong-Baker Faces Pain Rating Scale (WBFPR) and objective pain evaluation was done using the Faces, Leg, Activity, Consolability, Cry (FLACC) scale. Results: The outcome depicted a significant reduction in pain on injection for both objective and subjective evaluations in the thermo-mechanical device with an audio distraction group. Conclusions: Less pain on injection was observed, when a thermo-mechanical device was used with audio distraction for IANB procedures.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.13 no.2
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• pp.91-94
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• 2017

Hypercementosis is an excessive deposition of secondary cementum on the root of a tooth. It is mostly presented as a solitary lesion or in rare cases as a generalized type, but which is seldom recognized; typically it is discovered during regular dental X-ray. Increased thickness of cementum is not uncommon but generalized hypercementosis on impacted permanent teeth which may cause delayed eruption is rarely reported. This case report discusses a patient with cerebral palsy, epilepsy and mental retardation that presents multiple hypercementosis with delayed eruption. On intraoral examination, multiple retained primary molar teeth were found. As there was no any further symptoms, regular dental checkup had been done for several years. In 2015, a surgical opening was performed in the second molar area, but there was no specific change. Panoramic view showed multiple impacted permanent teeth with increased thickness of roots due to excessive deposition of cementum. Hypercementosis was also observed in the root of the erupted tooth. Several laboratory test results including hormone, urine, complete blood count test were reviewed. The patient was also diagnosed with subclinical hypothyroidism, impaired fasting glucose and had been taken valproic acid($Orfil^{(R)}$) for 10 years. However, none of them clearly explained generalized hypercementosis or delayed eruption. The patient is now 24 years old and regular dental checkups and radiographs are taken to confirm that there is no change in the lesion.

• Journal of the korean academy of Pediatric Dentistry
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• v.32 no.4
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• pp.589-594
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• 2005

With the progress of medical treatment techniques of bleeding control, dental care of the patient with hemophilia has become more convenient. So many surgical treatments can be performed with out-patient. 2 cases of intraoral surgical treatment of children, one with hemophilia 3, sever, the other with hemophilia A, severe. While the former was treated under general anesthesia, the latter was treated under local anaesthesia. There are principles : 1. When a patient with hemophilia need dental treatment, the dentist must consult to his physician, pediatrician, or hematologist before dental treatment. 2. When the dentist make a treatment plan, there should be consideration of the general condition, cooperation of the patient and risk of the treatment needed. 3. Minimize the number of times of coming for dental treatment so that reduce the times that need replacement therapy of coagulation factor. And during the treatment, dentist should care for infection and bleeding.

• Journal of Korean Clinical Nursing Research
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• v.26 no.2
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• pp.175-185
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• 2020

Purpose: This study was performed to develop a valid and reliable Pediatric Patient Classification System (PPCS). Methods: The study was conducted in a children's hospital which included various ward settings. Content validity was analyzed by Delphi method and to verify intraclass correlation reliability, 7 nurse managers and 29 staff nurses classified 216 patients. To verify construct validity, the staff nurses classified 216 patients according to PPCS comparing differences by age, days of stay, type of stay and medical department. Results: The developed PPCS has 12 categories, 55 nursing activities and 80 criterions. High agreement among nurses (r=.90) suggested substantial reliability. Construct validity was verified by comparing differences in age, days of stay, type of stay and medical department (p<.05). The entire patient group were classified to four groups using PPCS. Conclusion: The findings suggest that PPCS would be a useful tool for estimating nursing demands related to medications and the complexity of pediatric patients.