• The Journal of Korea Assosiation for Disability and Oral Health
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• v.8 no.2
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• pp.118-121
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• 2012

Down syndrome (DS) is a genetic disease known as trisomy 21. Congenital cardiac anomalies are present in about 40% of DS patients. Dental anomalies are also common among DS patients. In DS patients, canine impaction is 10 times more frequent and transposition of maxillary canine and first premolar is 50 times more common than in general population. A female DS patient with congenital heart disease was diagnosed as having a transposed impacted maxillary canine. Sectional fixed appliance with Nance holding arch was used for the orthodontic treatment. After space was regained for the eruption of the canine, orthodontic button was attached using flap operation with closed technique. Traction and alignment of the tooth followed. To prevent endocarditis, prophylactic antibiotics were prescribed for the recommended dental procedures. Total treatment time was 25 months and no complication was found.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.12 no.2
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• pp.77-81
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• 2016

The Dandy-Walker syndrome is a relatively rare congenital malformation occurring about one in 25,000-35,000 pregnancies. It is characterized by hydrocephalus, cystic dilatation of the fourth ventricle, complete or partial absence of the cerebellar vermis, and other various extra-CNS malformations. We report a dental caries treatment of a 4-year old girl with Dandy-Walker syndrome and partial trisomy 9q. The patient visited Seoul National University Dental Hospital due to multiple caries. The cause of multiple caries is mainly presumed as patient's eating habit caused by her general condition. She was still using milk bottle because she was having difficulties swallowing solid food due to breathing problem while eating. The treatment was performed under general anesthesia considering patient's condition; delayed development, very poor cooperation and respiratory problem. The dental procedure was successfully conducted and there were no postoperative complications. In consideration of her eating habit and oral hygiene problem, regular check appointment was recommended.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.12 no.2
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• pp.82-86
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• 2016

Lowe syndrome is X-linked gene disorder, characterized by cataracts, renal dysfunction and brain abnormalities. Oral healthcare of young patients with Lowe syndrome could be easily neglected due to the uncooperative behavior or other systemic condition of the child. We are presenting a case of successful treatment under general anesthesia with uncooperative child with Lowe syndrome. A 3-year old boy with Lowe syndrome visited Seoul National University Dental Hospital for multiple caries. He had been suffering from congenital cataract, medullary nephrocalcinosis and hypotonia. Multiple caries were observed from clinical and radiographic examination. Concerning behavior management problem and possibility of aspiration due to hypotonia, dental treatment under general anesthesia was planned. Left upper primary first molar was extracted to prevent further infection. Other teeth were treated with Stainless steel crown or composite resin restoration based upon the extent and the severity of dental caries. Under general anesthesia, dental procedure was carried out successfully and safely. Considering uncooperative behavior and other medical conditions of the patient, general anesthesia could be effective. Home oral care and periodic visit to dental clinic should be emphasized to the caregiver of patient with Lowe syndrome, considering the susceptibility of dental caries and other oral manifestation.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.15 no.1
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• pp.45-49
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• 2019

Bartter syndrome is an inherited renal tubular disorder characterized by hypokalemia, hypochloremic metabolic alkalosis, hyperreninemia, hyperprostaglandinism, and normal blood pressure. Bartter syndrome is classified by neonatal and classic type. Clinical manifestation of Bartter syndrome considered in dental treatment include tendency to dehydration, vomiting and erosion, attrition and abrasion of the teeth. A 2-year-4 month old boy with Bartter syndrome type III was referred to the Seoul National University Dental Hospital for dental evaluation and treatment. He showed hypokalemic hypochloremic metabolic alkalosis and had been treated with indomethacin and potassium chloride. He had hypocalcified teeth with or without multisurface caries lesions in all dentition and the erosion of maxillary teeth was detected. Dental procedure under general anesthesia was scheduled due to multiple caries and his medical condition. The dental procedure was successfully performed. This case suggests that electrolyte imbalances need to be treated prior to dental treatment and complete coverage restoration is necessary to protect the eroded teeth. An appropriate management plan for the patients with Bartter syndrome should include considerations of the need for close interaction with the pediatrician for pre- and post-operative care. General anesthesia may be recommendable to manage the patients having multiple caries with Bartter syndrome.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.15 no.1
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• pp.79-83
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• 2019

Treacher Collins syndrome(TCS) is an autosomal craniofacial development disorder which results from mutations in the gene TCOF1. Major features include midface hypoplasia, micrognathia, microtia, conductive hearing loss. Oral manifestations are characterized by cleft palate, shortened soft palate, malocclusion, anterior open bite and enamel hypoplasia. The purpose of this presentation is to describe the interesting aspects of dental treatment of a patient with TCS. A 6-year-old boy with TCS visited Seoul National University Dental Hospital for dental caries. Multiple caries was observed from clinical and radiographic examination. Because of multiple caries and behavior management ploblem, dental treatment under general anesthesia was planned. Treatment of posterior teeth was performed and some primary teeth were extracted. General anesthesia was induced and maintained with sevoflurane, nitrous oxide and oxygen. Under general anesthesia, successful dental procedure was done. Considering behavior management problem and medical condition of patient with TCS, general anesthesia can be useful.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.15 no.1
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• pp.65-69
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• 2019

Wolf-Hirschhorn syndrome(WHS) is a congenital disorder caused by deletions of the short arm of chromosome 4. The most common characteristics are mental and growth retardation, dietary disorder and craniofacial features with a characteristic 'Greek warrior helmet' appearance. The dental characteristic of WHS includes delayed development, tooth agenesis, clefts, microdontia, taurodontism, and severely worn dentition. The purpose of this case report is to describe the dental treatment of a patient with WHS. 3-year-old boy with WHS visited the Seoul National University Dental Hospital for dental treatment. He had difficulty with nasotracheal intubation because of craniofacial anomalies and also had poor oral hygiene due to a limitation of mouth opening and dietary disorder. Due to his airway problem, behavior management and severity of dental conditions, dental treatment was performed under general anesthesia. This case suggests general anesthesia can be chosen with WolfHirschhorn syndrome patients to safely care for their dental problems.

• Childhood Kidney Diseases
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• v.8 no.2
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• pp.205-213
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• 2004

Purpose: To evaluate the clinical manifestations of various glomerular diseases in children, a clinicopathological study was performed in 52 children who had renal biopsy. The type and relative incidence of the glomerular pathologies were analyzed, and the clinical predictability and usefulness of renal biopsy in glomerular diseases were assessed. Methods: Medical records of fifty two children with renal disease who had undergone percutaneous renal biopsy under ultrasonic guidance at Chungnam University Hospital from October 1995 to August 2003 were reviewed. In addition, we compared the clinical findings before renal biopsy with the pathological diagnosis. Results: The male to female ratio was 1.6:1 and they were $9.8\pm2.6$ years old on average. The chief complaints for biopsy were hematuria in 22 cases which was the most common (42.3%), proteinuria in 16 cases(30.8%), and hematuria & proteinuria(26.9%). Among the 22 cases of hematuria, there were 15 cases of gross hematuria(68.2%) and 7 cases of microscopic hematuria(31.8%). In terms of histopathologic diagnosis, most of them were primary glomerular diseases(84.6%), which included IgA nephropathy(28.8%), thin glomerular basement membrane disease(25.0%), focal segmental glomerulosclerosis(FSGS)(11.5%), membranous proliferative glomerulonephritis(7.7%), minimal change lesion(3.8%), acute poststreptococcal glomerulonephritis(3.8%) and membranous glomerulonephritis(3.8%). The clinical manifestations and pathologic diagnosis were not correlated. Conclusion: The clinical manifestations could not predict the pathological diagnosis. Therefore, renal biopsy would be inevitable in diagnosis of glomerular diseases for effective management and assessment of prognosis.

• Clinical and Experimental Pediatrics
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• v.45 no.7
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• pp.923-927
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• 2002

Congenital hepatic fibrosis is a relatively rare disease, characterized by bile ductular proliferation and prominent fibrosis in the portal area of liver resulting in portal hypertension. It is frequently associated with other abnormalities such as polycystic kidney, Caroli syndrome, cystic dysplasia of pancreas, intestinal lymphangiectasia, pulmonary emphysema, hemangioma, and cleft palate. We report here a case of congenital hepatic fibrosis associated with renal tubular ectasia in a 3-year-old girl, whose chief complaint was abdominal distension. Her liver function test did not reveal any abnormal findings. Hepatosplenomegaly and multiple dilated bile ducts were seen in the abdominal CT scaning. Esophageal varix was not detected by an endoscopic examination. Microscopically, diffuse portal fibrosis and widening with proliferation of blie ductules in the liver specimen and tubular ectasia in renal cortex were seen.

• Transactions of the Korean Society of Mechanical Engineers B
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• v.41 no.3
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• pp.153-160
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• 2017

Gait analysis has been conducted in various environments, but the biomechanics during the transition from uphill walking to downhill walking have not been reported. The purpose of this study is to investigate the knee and ankle joint kinematics and kinetics during walking on a triangle-shaped slope compared with those during level walking. Kinematic and kinetic data of eighteen participants were obtained using a force plate and motion capture system. The greater peak ankle dorsiflexion angle and moment and the peak knee extension moment were observed (p<0.05) during both uphill and downhill walking on the triangle-shaped slope. In summary, uphill walking on a triangle-shaped slope, which showed a peak knee flexion of more than $50^{\circ}$ with greater peak knee extension moment, could increase the risk of patellofemoral pain syndrome. Downhill walking on a triangle-shaped slope, which involved greater ankle dorsiflexion excursion and peak ankle dorsiflexion, could cause gastrocnemius muscle strain and Achilles tendon overuse injury.

• Journal of Veterinary Clinics
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• v.32 no.5
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• pp.436-439
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• 2015

Four cases of acute or acute-on-chronic kidney injury were referred for hemodialysis therapy. All cases had a history of uremic syndrome including refractory vomiting, anorexia, diarrhea and depression. They already had been treated with conventional therapies but the intractable clinical signs were remained without response of medications. Intermittent hemodialysis (IHD) sessions were executed in four cases. This study describes the clinical signs and biochemical features of four hemodialysis patients with dialysis adequacy indexed by Kt/V. This report introduced the estimation of hemodialysis adequacy method with clinical cases for establishment of standardization of hemodialysis therapy assess in dogs.