• Journal of the korean academy of Pediatric Dentistry
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• v.38 no.3
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• pp.270-275
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• 2011

Fibrous dysplasia is a developmental tumor-like condition that is characterized by replacement of normal bone by an excessive proliferation of cellular fibrous connective tissue intermixed with irregular bony trabeculae. Craniofacial lesions may cause facial pain, headache, cranial asymmetry, facial deformity, tooth displacement and visual or auditory impairment. In this case, a 2-year-9-month old boy who was diagnosed as the fibrous dysplasia showed delayed eruption on affected area. Teeth of left lateral dentition group have erupted completely but teeth of right lateral dentition group are erupting slowly. Eruption and maturation of affected teeth are in progress, so continuous observation is required presently. If the eruption state stops, surgical opening or forced eruption of the impacted teeth will be considered.

• Clinical and Experimental Pediatrics
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• v.51 no.5
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• pp.546-550
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• 2008

In this untreated classic phenylketonuria (PKU) case, mental retardation is severe; however, there have been individuals- like the mother of this case- who have escaped mental retardation and all the other potential sequelae of phenylketonuria, despite having high blood phenylalanine levels, and very poor dietary control. It appears that they have nearly normal brain phenylalanine levels despite high blood phenylalanine (Phe) levels. A number of studies have now demonstrated considerable variability in blood vs. brain phenylalanine levels in phenylketonuria patients. Outcome of phenylketonuria appears to be related to brain phenylalanine levels. We report a case of "undiagnosed" maternal phenylketonuria syndrome. A female infant had low birth weight (2,400 g) with microcephaly. We examined her family and discovered that her mother was an undiagnosed phenylketonuria patient with a borderline intelligence quotient (IQ). The infant's sister, six years old, was diagnosed with phenylketonuria at the age of four years was mentally retarded and had received an operation for cleft lip and palate. the sister had also had a low birth weight (2,300 g). Her sister and mother were compound heterozygotes (mother: R243Q/Y325X; sister: Y325X/P407S). The infant and father were heterozygous carriers (baby: R243Q/ -; father: P407S/ - ).

• Journal of Energy Engineering
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• v.25 no.3
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• pp.27-33
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• 2016

The heat recovery system that was applied in this study, is the energy-saving type that can produce the maximum cooling capacity less power in use. In order to have a more precise control function the temperature and humidity of the constant temperature and humidity machine, control algorithm is applied to designed a fuzzy PID controller, and the outside air compensation device (air-cooled) demonstrated excellent ability to dehumidify the moisture, $-20^{\circ}C$ in winter. High efficiency and the low-noise type sirocco fan operate quitely and designed to fit the bottom-up and top-down in accordance with the characteristics of equipment. as a result of experiment data, the conversion efficiency is 95% or more, power recovery time is within 5sec, stop delay time is within 30sec, pump down time is 10sec, pump delay time is 5sec, heating delay time is 5sec, temperature deviation is ${\pm}2^{\circ}C$ (cooling deviation: $2^{\circ}C$, Heating deviation : $2^{\circ}C$), humidity deviation is a ${\pm}5%$ (humidification deviation 3.0%, dehumidification deviation 3.0%). Recently, ubiquitous technology is important. so, the constant temperature and humidity machine designed to be able to remotely control to via the mobile phone, and more scalable to support MMI software and automatic interface. Further, the life of the parts and equipment is extended by the failure.

• Clinical and Experimental Pediatrics
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• v.51 no.1
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• pp.62-66
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• 2008

Purpose : In addition to epileptic seizures (ES), a variety of physiologic, organic and psychogenic disorders can manifest as paroxysmal behavioral events. Paroxysmal nonepileptic events (PNEs) are quite encountered in infants, young children, and adolescents. In a substantial proportion of cases, a careful history and examination will elucidate their nature. However, in other cases, it is necessary to differentiate PNEs from ES by video-electroencephalographic (EEG) monitoring. We report our experiences with PNEs in a group of children and adolescents who underwent video-EEG monitoring. Methods : From September, 2004 to June, 2006, one hundred thirty patients were monitored in the Pediatric Epilepsy Monitoring Units of Korea University Guro and Ansan hospitals. Their hospital charts were reviewed and video records of these events were analyzed. We observed all patients after video-EEG monitoring for more than 3 months. Results : Typical spells occurred during monitoring in 33 patients, not associated with a seizure pattern on EEG recordings. Two patients were diagnosed as frontal lobe epilepsy on basis of typical semiology and clinical characteristics, so 31 patients were documented to have PNEs finally. The mean age of patients was $7.2{\pm}5.8\;years$. The male to female ratio was 15 (48.4%) to 16 (51.6%). Among 31 patients, fifteen patients had associated disorders such as epilepsy, developmental delay, cerebral palsy, gastric ulcer, attention deficit hyperactivity disorder or depressive disorder. Somatoform disorder and factitious disorder was frequently seen in children more than 5 years old (P<0.05). Psychogenic disorder was more frequent in female (n=6) than in male (n=2) but there was no statistical significance (P>0.05). Conclusion : Our study suggests that video-EEG monitoring is an important diagnostic tool in the evaluation of paroxysmal behavioral events. With correct diagnosis of the PNEs, several unnecessary treatment could be avoided.

• Clinical and Experimental Pediatrics
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• v.48 no.7
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• pp.731-736
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• 2005

Purpose : Acute pyogenic osteomyelitis is uncommon in children. Delayed diagnosis and inappropriate treatment are leading to growth failure and deformation. We review the clinical manifestations and treatment of acute osteomyelitis in children according to age. Methods : A retrospective analysis was made of 32 patients who underwent antibiotic management or operation between Aug 1989 and Dec 2003 for acute pyogenic osteomyelitis in age from 0 to 15 years old. Results : The study group was composed of 21 boys and 11 girls. The subjects were divided into four groups according to age : 0-1 yr(n=6), 1-5 yr(n=11), 6-10 yr(n=8), and 11-15 yr(n=7). Nineteen cases were diagnosed in Winter. Femur was the most common infected site(37.5%). There were no predisposing factor in 17 patients, and 7 of 15 patients had trauma history. Sepsis was important predisposing factor in neonates. The chief complaints were pain, swelling and fever. S. aureus(61%) is the most common organism. Twenty-nine patients were treated with operation and concomitant antibiotics. Two cases had sequelae in follow-up period : One is avascular necrosis of femur and the other is discrepancy of leg length. Conclusion : In our review, because of poor prognosis in septic neonates, we recommend to treat actively neonatal sepsis and prevent or detect osteomyelitis early. Because most of patients were diagnosed and treated in orthopedic surgery, the rate of operation was too high. So, protocol for further evaluation and management of acute osteomyelitis in pediatric patients were needed.

• Clinical and Experimental Pediatrics
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• v.45 no.4
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• pp.466-472
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• 2002

Purpose : The diagnosis of Meckel's diverticulum is difficult and delayed because it presents with various clinical symptoms. We evaluated clinical, imaging and pathologic findings of Meckel's diverticulum to facilitate detection of Meckel's diverticulum in children. Methods : Review of clinical, imaging, surgical and pathological findings in 10 children aged 7 days to 14 years with Meckel's diverticulum during an 8-year period, 1993-2001, at Ewha Womans University Hospital was undertaken. Results : The male to female ratio was 2.3 : 1. The chief complaint was painless lower gastrointestinal( GI) bleeding; others were abdominal pain, abdominal distention and vomiting, in order of frequency. The diagonsis before surgery were Meckel's diverticulum in 5 patients, non-reducible intussusception in 3 patients and intestinal obstruction in 2 patients. The diverticulum was located between 35 cm to 70 cm proximal to the ileocecal valve. The length of the diverticulum ranged from 4 cm to 12 cm and 80% of it was within 5 cm. A Meckel scan($^{99m}Tc-pertechnetate$ scintigraphy) after cimetidine administration was done in 6 cases. All 5 cases that presented with lower GI bleeding had ectopic gastric mucosa confirmed on pathology. Out of 5 cases of ectopic gastric mucosa, only 4 cases were positive on the Meckel's scan. Conclusion : In cases of unexplained GI bleeding, obstruction, or inflammation diagnostic workup should be carried out to rule out Meckel's diverticulum. Laparoscopy, high resolution ultrasonography and computed tomography of the abdomen may be indicated in the assessment of pediatric patient with lower GI bleeding, especially in patients with suspected bleeding from Meckel's diverticulum showing negative Meckel's scan.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.17 no.3
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• pp.96-102
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• 2017

Glycogen storage disease type IX (GSD IX) is caused by deficiency of phosphorylase kinase which plays a role in breakdown of glycogen. Mutations in PHKA2 are the most common cause of GSD IX (GSD IXa). Clinical manifestations of GSD IXa include hepatomegaly, elevation of liver enzyme, growth retardation, fasting hypoglycemia, and fasting ketosis. However, the symptoms overlap with those of other types of GSDs. Here, we report Korean familial cases with GSD IXa whose diagnosis was confirmed by targeted exome sequencing. A 4-year old male patient was presented with hepatomegaly and persistently elevated liver enzyme. Liver biopsy revealed swollen hepatocyte filled with glycogen storage, suggesting GSDs. Targeted exome sequencing was performed for the differential molecular diagnosis of various types of GSDs. A hemizygous mutation in PHKA2 were detected by targeted exome sequencing and confirmed by Sanger sequencing: c.3632C>T (p.Thr121Met), which was previously reported. The familial genetic analysis revealed that his mother was heterozygous carrier of c.3632C>T mutation and his 28-month old brother had hemizygous mutation. His brother also had hepatomegaly and elevated liver enzyme. The hypoglycemia was prevented by frequent meals with complex carbohydrate, as well as cornstarch supplements. Their growth and development is in normal range. We suggest that targeted exome sequencing could be a useful diagnostic tool for the genetically heterogeneous and clinically indistinguishable GSDs. A precise molecular diagnosis of GSD can provide appropriate therapy and genetic counseling for the family.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.8 no.2
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• pp.150-156
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• 2005

Purpose: We divided the children diagnosed with intususception into three different age groups and compared the clinical symptoms and treatment outcomes in order to analysis diagnosis and treatment of Intusussception. Methods: A retrospective chart review was established of 159 patients diagnosed with intussuception who had been admitted to Department of Pediatrics and General Surgery, Bundang CHA hospital from January, 2000 to May, 2004. We divided the patients into three groups, according to their age. Those who were under five months of age (group I; 21 patients), those between 5~11 months of age (group II; 61 patients), and those older than 11 months of age (group III; 77 patients). Then we compared the age, sex, seasonal distribution of occurrence, the cause, the clinical features, radiologic review, the type of intusussception, surgical methods, recurrence rate and treatment outcomes, among these three groups. Results: On comparing the clinical symptoms and signs among the three groups, the most common major clinical symptom and sign was irritabiltity in all three groups. Vomiting with irritability was statistically more common in group I (p<0.05) and bloody stool was most frequent in group III. The average time taken until a diagnosis was made after the symptom onset was, 21 hours in group I, 20 hours in group II and 22 hours in group III. Which showed no significant difference. But there was a higher rate of delayed diagnosis in group I, which took over 48 hours until the diagnosis was made (group I; 23.8%, group II; 4.9%, group III; 7.8%). Simple abdominal X-rays showed more frequent instances of intestinal obstruction in group I (p<0.05). The primary treatment done was barium enema which showed a failure rate of 52.4% in group I, 26.2% in group II and 14.3% in group III. Showing the highest failure rate in group I. Conclusion: The youngest group had vague symptoms which lead to delayed diagnosis and more frequent surgical procedures. As so, we advocate the importance of further evaluation and close observation, considering intussusception in children with symptoms of irritability and vomiting, especially in the early infant group.

• The Journal of the Korea Contents Association
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• v.9 no.4
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• pp.247-253
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• 2009

PACS(Picture archiving communication system) is a system that enables medical images such as X -ray, CT, MRI, PET to be stored electronically viewed on computer screens so that doctors and other authorized people can access search the information as needed. But if they are not in hospital area for example on holiday or at night, that are not able to access the PACS system instantly. We have to solve this problem for more efficient patient care. So we try to suggest a method that use the PDA system that wireless LAN and CDMA cellular phone are equipped. This system may help to access easier to PACS system regardless of the location and can also attribute the development of telemedicne.

• Tuberculosis and Respiratory Diseases
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• v.64 no.2
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• pp.144-148
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• 2008

Tracheomegaly is a distinctive condition that presents with marked dilation of the trachea. Spontaneous pneumomediastinum is the result of alveolar rupture with dissection of the airway along the bronchus and into the mediastinum. Tracheomegaly and recurrent spontaneous pneumomediastinum are rare complications of pulmonary fibrosis when combined with rheumatoid arthritis. We present a case of tracheomegaly and recurrent spontaneous pneumomediastinum that was precipitated by repeated respiratory infection and chronic cough in a patient with pulmonary fibrosis that was associated with rheumatoid arthritis.