• The Journal of Korean Medicine
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• v.25 no.4
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• pp.129-138
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• 2004

Objective : Treatments for patients with mental retardation and pervasive developmental disorders are not curative, and are designed to help those with disabilities adjust to their environments and daily demands. As clinicians, the present authors tried to find agents with potentially curative properties. Among the numerous herbal formulations available, we chose and assessed Gami-jiwhang-tang (GJT) in the hope that it would improve cognitive development of children. Methods : Subjects were typically-developing healthy, 7- to 8-year-old boys and girls living in Seoul, Korea. The experimental group took GJT for six weeks and was followed up six weeks after discontinuation of GJT. The control group was assessed at the same intervals but did not receive placebos. To measure the effects of GJT, neuropsychological tests and intelligence test were taken before commencing GJT and twelve weeks later. Resulets and Conclusion : For all of the ANOVAs, the treatment by time interaction terms was not significant. However, the experimental group showed the tendency to be progressed in most subscales compared with the control group, especially on performance intelligence, visual organization, and verbal fluency. Conclusion : Although GJT failed to reveal significant improvement in cognition, we remain hopeful about the compound and believe that it should be evaluated by a double-blind, placebo-controlled trial in the future.

• Clinical and Experimental Pediatrics
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• v.57 no.5
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• pp.240-244
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• 2014

Pseudohypoparathyroidism type Ia (PHP Ia) is a disorder characterized by multiform hormonal resistance including parathyroid hormone (PTH) resistance and Albright hereditary osteodystrophy (AHO). It is caused by heterozygous inactivating mutations within the Gs alpha-encoding GNAS exons. A 9-year-old boy presented with clinical and laboratory abnormalities including hypocalcemia, hyperphosphatemia, PTH resistance, multihormone resistance and AHO (round face, short stature, obesity, brachydactyly and osteoma cutis) which were typical of PHP Ia. He had a history of repeated convulsive episodes that started from the age of 2 months. A cranial computed tomography scan showed bilateral calcifications in the basal ganglia and his intelligence quotient testing indicated mild mental retardation. Family history revealed that the patient's maternal relatives, including his grandmother and 2 of his mother's siblings, had features suggestive of AHO. Sequencing of the GNAS gene of the patient identified a heterozygous nonsense mutation within exon 11 (c.637 C>T). The C>T transversion results in an amino acid substitution from Gln to stop codon at codon 213 ($p.Gln213^*$). To our knowledge, this is a novel mutation in GNAS.

• Journal of The Korean Dental Society of Anesthesiology
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• v.14 no.1
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• pp.57-62
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• 2014

Background: The aim of this study was to analyze outpatient anesthesia for dental treatment in handicapped patients with behavior disorder in order to use data for carrying out better and safe anesthetic management. Methods: The data were drawn from the 100 patients with behavior disorder who visited CNUDH dental clinic for disabled based on anesthesia record to investigate patient's systemic condition, cooperative level, anesthesia method according to patients cooperation, and side effects after recovery time. Results: Mental retardation (58%) is the most reason to choose general anesthesia. The methods of induction according to cooperative level are intravenous propofol injection in 22 cases and inhalation of sevoflurane in 78 cases. Induction time of anesthesia were within 10 seconds in cases of propofol induction and average $48.8{\pm}18.5$ seconds in cases of inhalation induction. The time spent on dental treatment was average $3.2{\pm}1.1$ hours. After the end of treatment, average time to move from unit chair to recovery bed, to recliner, and to discharge from hospital are $10.4{\pm}5.1$, $36.9{\pm}17.1$ and $72.4{\pm}16.0$ minutes, respectively. During recovery, there are nausea with 9%, vomiting with 4%, dizziness with 2%, finger injury with 1%. Conclusions: This study showed our successful anesthetic outcomes without any severe side effects or complications. Through this study, it will be used for safe anesthetic management as useful reference data.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.8 no.1
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• pp.22-25
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• 2012

Hypoxic ischemic encephalopathy(HIE) is a condition that occurs when the entire brain is deprived of an adequate oxygen supply and associated in most cases with oxygen deprivation in the neonate due to birth asphyxia. Children with HIE develop problems such as cerebral palsy, mental retardation, learning difficulties, and other disabilities. We hereby report the case of treating a child patient with HIE whose chief complaint of severe lingual ulceration. A 12-month-old boy referred to our hospital to treat ulcers of ventral surface of tongue. He has been suffering from lingual ulceration due to persistent tongue thrusting. He has bitten his own tongue involuntary. The ulceration was apparently seemed to be not healed because of the constant irritation by lower incisors. So we designed tongue protector to prevent lingual ulcers. The appliance was proved to be effective to reduce tongue trauma by lower incisors and periodic follow-up is needed.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.5 no.2
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• pp.87-91
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• 2009

The purpose of this study were to investigate the chief complain and dental treatment needs in handicapped patient. This study examines treatment records of 1025 patients in free dental clinic for handicapped patients during 10 years from 1999 to 2008. The results were as follows : 5.8 average visit per patient; mean patient age was 25; 544 patients was younger than 20. Handicapped patients classified according to types of disability. Crippled disorder were 19.1%, brain disorder were 4.5%, visual disorder were 3.1%, auditory disorder were 4.1%, speech disorder were 0.9%, mental retardation were 67.1%, and developmental disorder were 25.1%. Performed treatments were 322 scaling, 13 fluoride varnish, 727 preventive resin restoration, 1296 resin restoration, 600 amalgam restoration, 46 GI restoration, 612 extraction, 289 pulp treatment of primary teeth, 75 pulp treatment of permanent teeth and 138 stainless steel crown restoration. Many handicapped patients have some difficulty to dental treatment. They have limited access to dental care, which is compounded by a shortage of skilled dental professionals who are willing to treat these population and financial problems.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.10 no.1
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• pp.38-42
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• 2014

A tracheostomy tube serves as airway management for patients whose respiration is impeded due to inflammation, tumor, or traumatic events. If the patients who have tracheostomy tube, visit dental clinic for dental treatments, we should consider the underlying general condition of patients and then make treatment plans according to their state. A 22-Year old male patient, who had tracheostomy tube on his neck, came to our department for comprehensive dental treatment. Mild mental retardation was observed and he was taking anti-convulsant drugs for the prevention of epileptic seizure. Multiple advanced dental caries, hopeless teeth, and impacted third molars were also observed by clinical and radiographic examination. Due to the risk of epileptic seizure and low cooperativity to tolerate the treatment, general anesthesia was recommended by physician, and the anti-convulsant drug was administrated during procedure. In this case, we aimed to report the multidisciplinary approach for the dental treatment of patient having a tracheostomy tube.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.9 no.1
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• pp.25-29
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• 2013

Most of disabled people face hardness in caring their general oral hygiene by themselves. So that, they are once involved in dental caries or periodontal disease, they have much bigger chance of aggressive progress. Therefore preventive dentistry is more important to disabled one than non-disabled. They need to be checked with routine periodic dental examination and by that, oral disease must be found at initial stage. We selected 37 patients from newly visited 237 patients who had dental treatment at the free dental clinic, Gangnam district, Seoul, between 2000 and 2001. This study is a comparative evaluation of first 2 years' treatment records of selected 37 patients (male 28, female 9) with that of their last 2 years. 24 of 37 have mental retardation, 9 have autistic disorder, 2 have auditory disorder, 1 has brain disorder and 1 has crippled disorder. Their dental treatment records categorized by 4 level; score 4 stands for endodontic treatment, score 3 stands for general conservative treatment, score 2 stands for preventive treatment, such as sealant and score 1 stands for routine check. In first 2 years of treatment, average score was 2.85 which score means approximately general conservative treatment. And that of last 2 years was 1.44, which means routine check. In early time of their visit, they will receive the treatment due to their chief complaint. And after that early time, they can have a routine check so that they can be treated before the disease get worse. The patient's oral health can remain decent only by a simple treatment.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.9 no.2
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• pp.127-130
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• 2013

Hypoxic ischemic encephalopathy (HIE) is an important cause of permanent damage to central nervous system cells, most cases with oxygen deprivation in the nenonate due to birth asphyxia. Survival children with HIE develop problems such as cerebral palsy, mental retardation, learning difficulties, and other permanent neuro-developmental disabilities. This article report two cases of the children with HIE who had be traumatized on their teeth from oropharyngeal airway (OPA) in the emergency situation. The patients with the disease accompanying seizure or convulsion, needs special consideration for the prevention from dental trauma in emergency airway management.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.3 no.2
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• pp.91-96
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• 2007

Cleidocranial dysplasia(CCD) is a congenital genetic disorder of skeletal and dental abnormality, which is mesodermal dysfunction influencing many tissues and organs, CCD was reported by Morand at first in 1766, And later, it was named cleidocranial dysostosis, cleidocranial dysplasia, Marie-sainton syndrome and mutational dysostosis. It is autosomal dominant disorder and there is no prevalence between man and woman. Until recent days, mutation of Runx2 in chromosome6p21 has known to be a main factor causing CCD. The specific clinical features of CCD are aplasia or hypoplasia of one or both clavicles and incomplete closing of fontanels and cranial sutures. Dental manifestations include retention of deciduous teeth, delayed eruption of permanent teeth, supernumerary teeth and cyst. Because there is no mental retardation and physical disability in CCD patients, they usually can not recognize their dental abnormality by the time of abolescence. So, after exfoliation of deciduous teeth, they usually live with edentulous status. It usually drives CCD patients to suffer from esthetic and functional problem. For this reason, CCD patients must be early diagnosed and improved in their appearance as well as masticatory function. So, surgical removal of supernumerary teeth and orthodontic eruption of the natural permanent teeth at adequate time is necessary.

• Journal of the Korean Academy of Child and Adolescent Psychiatry
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• v.16 no.2
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• pp.153-159
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• 2005

Autistic disorder and other PDD are currently viewed as a largely genetically determined neurodevelopmental disorder, although its underlying biological causes remain to be established. In this review, we examine the available neurodevelopmental literature on autistic disorder and discuss the findings that have emerged. Typical neuropathological observations are rather consistent with respect to the limbic system (increased cell packing density and smaller neuronal size), the cerebellum (decreased number of Purkinje cells) and the cerebral cortex ($>50\%$ of the cases showed features of cortical dysgenesis). However, most of the reported studies had to contend with the problem of small sample sizes, the use of quantification techniques, not free of bias and assumptions, and high percentages of autistic subjects with comorbid mental retardation or epilepsy. Furthermore, data from the limbic system and on age-related changes lack replication by independent groups. It is anticipated that future neuropathological studies held great promise, especially as new techniques such as design-based stereology and gene expression are increasingly implemented and combined, larger samples are analysed, and younger subjects free of comorbidities are investigated.