• Clinics in Shoulder and Elbow
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• v.2 no.2
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• pp.209-213
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• 1999

It is well known that tardy ulnar nerve palsy occurs with cubitus valgus deformity as a late complication after a nonunion of lateral condyle fracture of the humerus in childhood. On the other hand, cubitus varus deformity often results from malunion of supracondylar fractures of the humerus. However, reports of tardy ulnar nerve palsy in cubitus varus deformity are few. We report a patient with bilateral cubitus varus deformities with bilateral tardy ulnar nerve palsy which was confirmed with EMG. She was treated by step cut osteotomy(DeRosa and Graziano) with sub­cutaneous anterior transposition of ulnar nerve.

• Korean Journal of Child Studies
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• v.31 no.4
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• pp.49-60
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• 2010

This study sought to investigate and assess the development patterns of children, aged from 3 to 5, by means of a longitudinal approach. The children's developmental patterns are classified according to five curriculum areas; physical health, social skills, expression, language, and exploration-. The developmental patterns are analyzed in detail according to the observation period, children's ages, and their genders. The subjects consisted of 108 children in A city. A research assistant was asked to observe and keep records of the children's behaviors at three distinct times -early, middle, and late in the school year. The 'observational scale for children' was used as the measurement tool. The data which was thus collected was then subject to statistical analysis. The major findings of the study are as follows. First, there were significant differences in all five curriculum areas according to the children's age and observation period. That is, five-year-old children showed higher scores than three- and four-year-old children. Second, there were significant differences in the social development within five curriculum areas according to the children's gender and the observation period. That is, girls exhibited higher scores than boys.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.22 no.1
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• pp.1-8
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• 2022

Long-chain fatty acid oxidation disorders (LC-FAOD) are an autosomal recessive inherited rare disease group that result in an acute metabolic crisis and chronic energy deficiency owing to the deficiency in an enzyme that converts long-chain fatty acids into energy. LC-FAOD includes carnitine palmitoyltransferase type 1 (CPT1), carnitine-acylcarnitine translocase (CACT), carnitine palmitoyltransferase type 2 (CPT2), very long-chain acyl-CoA dehydrogenase (VLCAD), long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD), and trifunctional protein (TFP) deficiencies. Common symptoms of LC-FAOD are hypoketotic hypoglycemia, cardiomyopathy, and myopathy. Depending on symptom onset, the disease can be divided as neonatal period, late infancy and early childhood, adolescence, or adult onset, but symptoms can appear at any time. The neonatal screening test (NBS) can be used to identify the characteristic plasma acylcarnitine profiles for each disease and confirmed by deficient enzyme analysis or molecular testing. Before introduction of NBS, the mortality rate of LC-FAOD was very high. With NBS implementation as routine neonatal care, the mortality rate was dramatically decreased, but severe symptoms such as rhabdomyolysis recur frequently and affect the quality of life. Triheptanoin (Dojolvi^®), the first drug for pediatric and adult patients with molecularly confirmed LC-FAOD, has recently been approved by the US Food and Drug Administration in 2020. In this review, the diagnosis of LC-FAOD and treatment including triheptanoin are summarized.

• Clinical and Experimental Pediatrics
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• v.46 no.5
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• pp.495-499
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• 2003

Purpose : Eosinophil is one of the important inflammatory cell involved in the airway inflammation in childhood asthma. It has been demonstrated that markers of eosinophil activation, including eosinophil cationic protein or eosinophil protein X(EPX), are increased in childhood asthma. Furthermore, they are related to disease activity and are assumed to be helpful in monitoring the treatment effect as urinary EPX(U-EPX) can be obtained easily and in a noninvasive way in children of all ages. Methods : Twenty-five children(22 male and three female) aged $11.87{\pm}3.82$ years with stable asthma were challenged with methacholine and urine was collected from each child during the following periods; before methacholine challenge test(MCT); 0-3 hr after the end of MCT; 4-7 hr after the end of MCT; and 8-24 hr after the end of MCT. Bronchial reactivity was determined by using Dosimeter( Jeager, Germany) with serially diluted methacholine from 0.05 to 25.0 mg. The $FEV_1$ less than 80% of baseline value were classified into positive MCT. U-EPX was measured with a sensitive and specific radioimmunoassay(Pharmacia & Upjohn AB, Uppsala, Sweden). Results were expressed as ${\mu}gEPX/mmol$ creatinine. Results : An early airway response after MCT was associated with an increase of U-EPX excretion for 0-3 hr after methacholine inhalation in comparison with beseline values. Most subjects showed a small increase in U-EPX excretion during late asthmatic response for 4-7 hr, which then decreased to normal level in 8-24 hr. Also, a tendency for a higher increase of U-EPX was associated with a lower threshold of methacholine challenge and a longer duration of asthma. Conclusion : Measurement of EPX in urine is a noninvasive and easy method to assess the severity of airway inflammation in asthmatic children. It may be a helpful index of the events underlying the airway inflammatory responses during nonspecific bronchial challenge, and in monitoring asthma management.

• Asian Pacific Journal of Cancer Prevention
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• v.15 no.18
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• pp.7863-7867
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• 2014

Background: Retinoblastoma is a rare type of cancer that usually develops in early childhood. If left untreated it can cause blindness and even death. The aim of this study is to determine sociodemographic and clinical features of retinoblastoma patients and also to determine the treatment pattern and outcome in Malaysia. Materials and Methods: Data for this study were retrieved from the Retinoblastoma Registry of the National Eye Database (NED) in Malaysia. Hospital Kuala Lumpur, Hospital Umum Kuching, Sarawak and Hospital Queen Elizabeth, Kota Kinabalu were the major source data providers for this study. Data collected in the registry cover demography, clinical presentation, modes of treatment, outcomes and complications. Results: The study group consisted of 119 patients (162 eyes) diagnosed with retinoblastoma between 2004 and 2012. There were 68 male (57.1%) and 51 (42.9%) female. The median age at presentation was 22 months. A majority of patients were Malays (54.6%), followed by Chinese (18, 5%), Indians (8.4%), and indigenous races (15.9%). Seventy six (63.8%) patients had unilateral involvement whereas 43 patients had bilateral disease (36.1%). It was found that most children presented with leukocoria (110 patients), followed by strabismus (19), and protopsis (12). Among the 76 with unilateral involvement (76 eyes), enucleation was performed for a majority (79%). More than half of these patients had extraocular extension. Of the 40 who received chemotherapy, 95% were given drugs systemically. Furthermore, in 43 patients with bilateral involvement (86 eyes), 35 (41%) eyes were enucleated and 17 (49%) showed extraocular extension. Seventy-two percent of these patients received systemic chemotherapy. The patients were followed up 1 year after diagnosis, whereby 66 were found to be alive and 4 dead. Sixteen patients defaulted treatment and were lost to follow-up, whereas follow-up data were not available in 33 patients. Conclusions: Patients with retinoblastoma in this middle-income Asian setting are presenting at late stages. As a result, a high proportion of patients warrant aggressive management such as enucleation. We also showed that a high number of patients default follow-up. Therefore, reduction in refusal or delay to initial treatment, and follow-up should be emphasized in order to improve the survival rates of retinoblastoma in this part of the world.

• Pediatric Infection and Vaccine
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• v.18 no.1
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• pp.61-67
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• 2011

Purpose : This study was performed to investigate the epidemiologic characteristics of human bocavirus (HBoV)-associated lower respiratory tract infections (LRTIs) in children. Methods : Nasopharyngeal aspirate samples were obtained from 658 children who had been hospitalized for LRTIs in Seoul National University (SNU) Children's Hospital and SNU Bundang Hospital from March 2000 to September 2005. Multiplex RT-PCR was performed to detect 11 respiratory viruses including respiratory syncytial virus, adenovirus, rhinovirus, parainfluenza viruses 1 and 3, influenza viruses A and B, human metapneumovirus, HBoV, human coronavirus (HCoV) OC43/ 229E, and HCoV-NL63. Clinical data were reviewed retrospectively. Results : Overall, respiratory viruses were detected in 325 (49.4%) among 658 patients. HBoV was detected in 62 cases (9.4%) and was responsible for 19.1% of virus-positive cases. HBoV was prevalent among infants and young children aged from 3 months to 5 years with the mean age of 25.3 months. Co-detection of HBoV and other respiratory viruses was observed in 37.1% which is significantly higher than average co-detection rate (12.3%) among overall virus-positive cases (P=0.000). HBoV was identified mainly in late spring and early summer from May to July. Conclusion : This study describes epidemiologic features of HBoV in Korean children compared with those associated with other respiratory viruses. HBoV was prevalent among LRTIs in childhood, especially in late spring and early summer season in Korea.

• Journal of the korean academy of Pediatric Dentistry
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• v.26 no.1
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• pp.146-150
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• 1999

With the improved cure rates for childhood malignant conditions in the past decade, late effects of cancer therapy must be recognized to minimize their impact on the quality of life in long-term survivors. Chemoradiation therapy is a major part of pediatric oncology treatment and is implicated in causing tooth agenesis, microdontia, root shortening, early apical closure, and coronal hypocalcification. Dental development may be affected by illness, trauma, chemotherapy, or radiation therapy at any point prior to complete maturation. Treatment given during the first 3.5 years of life was more likely to affect the dental lamina and crown formation and result in a small tooth. Dental treatment affected by chemoradiation damage to developing teeth includes orthodontic tooth movement, prosthetic abutment consideration, periodontal health, space maintenance, requirement for home fluoride regimens to protect hypomineralized teeth, and enodontic procedures. Dental abnormalities are common in patients treated for cancer, and these children require aggressive dental follow-up. Meticulous surveillance may facilitate detection of abnormalities, enabling the dental practitioner to intervene earlier in promoting a more aggressive regimen of oral care, thus reducing the morbidity associated with dental sequelae of oncotherapy, specifically periodontal disease and malocclusion. In this case, we report microdontia of all permanent second premolar and second molar in an 8 year old boy treated with chemotherapeutic agents during period of active dental development(14 months to 38 months of age).

• The Korean Journal of Food And Nutrition
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• v.27 no.6
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• pp.979-989
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• 2014

The purpose of this study was to investigate the association between dietary behavior and temperament & character in preschool children, and to offer basic data that can be applied for nutrition education and counseling. A total of 211 parents of preschool children aged 3~5 years performed the Korean version of Preschool Temperament and Character Inventory (K-psTCI), a questionnaire based on Cloninger's seven-factor model of personality, along with a questionnaire about the dietary behaviors of their children. K-psTCI represented seven factors such as harm avoidance (HA), novelty seeking (NS), reward dependence (RD), persistence (P), self-directedness (SD), cooperativeness (CO), and self-transcendence (ST). The subjects were divided into either the high rank group or low rank group based on the mean score of each factor. The high rank group of HA showed significantly less physical activity and less appetite than the low rank group of HA. The children in the high rank of NS were more likely to have picky eating and a late night snack. The children in the low rank of SD or CO were more likely to have undesirable dietary behaviors, such as picky eating, too much snacking, and lower appetite than those in the high rank of SD or CO. In conclusion, individual temperament & character in preschool children may be associated with their dietary behavior, and understanding temperament & character in children may be important facts to screen and to develop an effective nutrition education program for children.

• Childhood Kidney Diseases
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• v.8 no.2
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• pp.250-255
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• 2004

Wilson's disease is an autosomal recessive disorder characterized by degenerative changes in the brain, liver, and cornea. Treatment includes D-penicillamine, trientine, and zinc sulfate. D-penicillamine has been used frequently as first line therapy for Wilson's disease. However, nephrotoxicity can occur after D-penlcillamlne treatment. Among them membranous glomerulopathy is the most common histological abnormality but minimal change lesions have also been reported. Nephrotic syndrome is a late complication of D-penicillamine treatment but very rarely can occur within 2 months after treatment of D-penicillamine. We report the early development of minimal change nephrotic syn,frome in a 3-year-old'girl with Wilson's disease 3 weeks after initiation of D-penicillamine.

• Childhood Kidney Diseases
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• v.19 no.2
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• pp.112-117
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• 2015

Purpose: The incidence of acute poststreptococcal glomerulonephritis (APSGN) in Korea has changed. This study aimed to evaluate the epidemiological and clinical changes of APSGN observed in a single Korean institution over two decades. Methods: We retrospectively analyzed the data of 99 children (0-15 years of age) who were admitted to our institution with APSGN between 1987 and 2013. The patients were selected based on the depression of serum complement 3 (C3, <70 mg/dL) and elevated titer of antistreptolysin O (ASO, >250 IU/dL) as evidence of previous streptococcal infection. Results: In the 99 patients, the mean age was $8.3{\pm}2.7$ years, and the male-to-female ratio was 2.2:1 (66:30). The annual number of cases fluctuated markedly, and most cases were observed during the late autumn and winter months. However, there have been few cases reported in the past 5 years. Clinical manifestations at presentation, including hypertension and generalized oedema, and the duration of hospitalization were higher and longer in patients admitted during the first half of the study period than during the most recent half-period, suggesting a more severe clinical course in the former group. Conclusions: APSGN has become a rare disease in Korea with a trend towards a less severe clinical course. This finding suggests that the prevalence of infection-related immune-mediated diseases could change over-time, together with environmental and possibly pathogen-host relationship changes.