• Journal of the korean academy of Pediatric Dentistry
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• v.11 no.1
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• pp.91-101
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• 1984

The purpose of this study is to determine what influence the lesion and the premature loss of mandibular 1st deciduous molar have on the eruption of its successor, and the author devided 580 cases of orthopantomograms from age 3 to 9 years old children into 3 groups: Control Group: both side normal Study I Group: one normal and the other lesion. Study II Group: one normal and the other premature loss before 8 years old. and observed the amount of differences and the relative position in eruption between study-tooth successor and normal-tooth successor, The following results were obtained: 1. The differences in eruption between right and left mandibular 1st primary molars of Study Group were greater than those of Control Group. 2. The successors of lesioned-teeth showed more accelerated eruption than the antimeres in 40.9% and more delayed, in 22.7%. 3. The successors of premature lost-teeth showed more accelerated eruption than the antimeres in 61.7% and more delayed, in 4.3%.

• Clinical and Experimental Pediatrics
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• v.59 no.sup1
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• pp.25-28
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• 2016

Phelan-McDermid syndrome is a rare genetic disorder caused by the terminal or interstitial deletion of the chromosome 22q13.3. Patients with this syndrome usually have global developmental delay, hypotonia, and speech delays. Several putative genes such as the SHANK3, RAB, RABL2B, and IB2 are responsible for the neurological features. This study describes the clinical features and outcomes of Korean patients with Phelan-McDermid syndrome. Two patients showing global developmental delay, hypotonia, and speech delay were diagnosed with Phelan-McDermid syndrome via chromosome analysis, fluorescent in situ hybridization, and multiplex ligation-dependent probe amplification analysis. Brain magnetic resonance imaging of Patients 1 and 2 showed delayed myelination and severe communicating hydrocephalus, respectively. Electroencephalography in patient 2 showed high amplitude spike discharges from the left frontotemporoparietal area, but neither patient developed seizures. Kidney ultrasonography of both the patients revealed multicystic kidney disease and pelviectasis, respectively. Patient 2 experienced recurrent respiratory infections, and chest computed tomography findings demonstrated laryngotracheomalacia and bronchial narrowing. He subsequently died because of heart failure after a ventriculoperitoneal shunt operation at 5 months of age. Patient 1, who is currently 20 months old, has been undergoing rehabilitation therapy. However, global developmental delay was noted, as determines using the Korean Infant and Child Development test, the Denver developmental test, and the Bayley developmental test. This report describes the clinical features, outcomes, and molecular genetic characteristics of two Korean patients with Phelan-McDermid syndrome.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.24 no.3
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• pp.288-294
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• 2021

Purpose: Despite aggressive medical and nutritional management, patients with methylmalonic acidemia (MMA) often suffer from multi-organ damage. Early deceased donor liver transplantation (DDLT) has emerged as an intervention to prevent disease progression. We investigated the efficacy of living donor LT (LDLT) with a potential carrier of MMA and a small volume of graft in patients with MMA as an alternative to DDLT. Methods: Of five patients (three male, two female; median age 5.7 years; range, 1.3-13.7 years), four underwent carrier LDLT, while one underwent non-carrier auxiliary LDLT. All patients received pre- and post-LT continuous renal replacement therapy and were provided with minimal restriction diet according to serum MMA level after LT. MMA levels in the serum and urine, the incidence of metabolic crisis, and clinical findings before and after LT were compared. Results: The survival rate was 100% during 2.2 years of follow up period after LT. In all five cases, MMA titer in the serum after transplantation decreased with less restrictive diet. Metabolic crisis was not observed during the follow-up period. In addition, no patient showed progression of severe renal impairment requiring hemodialysis. Progression of delayed cognitive development was not observed. Social functioning with improved neuropsychiatric development was observed. Conclusion: This study showed that LDLT achieved improved quality of life with less restrictive diet, therefore it could be a feasible alternative option to DDLT for the treatment of patients with MMA, even with an auxiliary LT.

• Asian Pacific Journal of Cancer Prevention
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• v.17 no.4
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• pp.1661-1675
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• 2016

Objectives: Can addition of neurokinin-1 receptor antagonists (NK1-RAs) be considered as an ideal strategy for the prevention of chemotherapy-induced nausea and vomiting (CINV)? Researchers differ on this question. Materials and Methods: Electronic databases were searched for randomized control trials (RCTs) that evaluated the effectiveness and safety of NK1-RAs in preventing CINV. The primary end point was complete response (CR) in the acute, delayed, and overall phases after chemotherapy. Subgroup analyses evaluated the types of NK1-RAs, routines of administration, types of malignancies, regimens used in combination with NK1-RAs, and age of patients included in the studies. The incidences of different types of adverse events were also extracted to estimate the safety of NK1-RAs. Results: A total of 38 RCTs involving 13,923 patients were identified. The CR rate of patients receiving NK-RAs was significantly higher than patients in the control groups during overall phase (70.8% vs 56.0%, P<0.001), acute phase (85.1% vs 79.6%, P<0.001), and delayed phase (71.4% vs 58.2%, P<0.001). There were three studies including patients of children or adolescents, the CR rate was also significantly higher in the treatment group (overall phase: OR=2.807, P<0.001; acute phase: OR=2.863, P =0.012; delayed phase: OR=2.417, P<0.001). For all the other outcomes, patients in the NK1-RAs groups showed improvements compared to the control groups (incidence of nausea: 45.2% vs 45.9%, P<0.001; occurrence of vomiting: 22.6% vs 38.9%, P<0.001; usage of rescue drugs: 23.5% vs 34.1%, P<0.001). The pooled side effects from NK1-RAs did not significantly differ from previous reports and the toxicity rates in patients less than eighteen years old also did not diff between the two groups (P=0.497). However, we found that constipation and insomnia were more common in the patients of control groups, whereas diarrhea and hiccups were more frequently detected in patients receiving NK1-RAs. Conclusions: NK1-RAs improved the CR rate of CINV. They are effective for both adults and children. The use of NK1-RAs might be associated with the appearance of diarrhea and hiccups, while decreasing the possibility of constipation and insomnia.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.22 no.4
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• pp.358-368
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• 2019

Purpose: Pediatric Crohn's disease (CD) is directly related to growth and has a high probability of requiring surgical intervention(s); therefore, more active treatment for CD is required for children. This study investigated the impact of biologics on growth and disease course associated with surgery. Methods: This was a retrospective cohort study involving patients diagnosed with CD at the Seoul National University Children's Hospital (Seoul, Korea) between January 2006 and October 2017. The aim was to determine the characteristics of pediatric patients with CD and whether biologics affected growth and the surgical disease course. Results: Among patients who underwent surgery for CD, the mean number of operations per patient was 1.89. The mean time from initial diagnosis to surgery was 19.3 months. The most common procedure was fistulectomy (34%), followed by incision and drainage (25%). In all patients, the use of biologics increased the height (p=0.002) and body mass index (BMI) (p=0.005). Among patients who underwent surgery, height (p=0.004) and BMI (p=0.048) were increased in the group using biologics. Patients who used biologics exhibited a low operation rate only within 2 years after diagnosis, with no differences thereafter (p=0.027). Conclusion: Although biologics could not mitigate the operation rate in pediatric patients who underwent surgery for CD, biological therapy delayed disease progression within 2 years of disease onset. Additionally, biologics conferred growth and BMI benefits in this window period. Therefore, it may be helpful to use biologics for optimal growth in pediatric patients with a high probability of undergoing future surgery.

• Journal of Ginseng Research
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• v.48 no.1
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• pp.68-76
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• 2024

Background: Although the survival outcomes of childhood cancer patients have improved, childhood cancer survivors suffer from various degrees of immune dysfunction or delayed immune reconstitution. This study aimed to investigate the effect of Korean Red Ginseng (KRG) on T cell recovery in childhood cancer patients who underwent autologous hematopoietic stem cell transplantation (ASCT) from the perspective of inflammatory and senescent phenotypes. Methods: This was a single-arm exploratory trial. The KRG group (n = 15) received KRG powder from month 1 to month 12 post-ASCT. We compared the results of the KRG group with those of the control group (n = 23). The proportions of T cell populations, senescent phenotypes, and cytokine production profiles were analyzed at 1, 3, 6, and 12 months post-ASCT using peripheral blood samples. Results: All patients in the KRG group completed the treatment without any safety issues and showed a comparable T cell repopulation pattern to that in the control group. In particular, KRG administration influenced the repopulation of CD4⁺ T cells via T cell expansion and differentiation into effector memory cell re-expressing CD45RA (EMRA) cells. Although the KRG group showed an increase in the number of CD4⁺ EMRA cells, the expression of senescent and exhausted markers in these cells decreased, and the capacity for senescence-related cytokine production in the senescent CD28^- subset was ameliorated. Conclusions: These findings suggest that KRG promotes the repopulation of CD4⁺ EMRA T cells and regulates phenotypical and functional senescent changes after ASCT in pediatric patients with cancer.

• Clinical and Experimental Pediatrics
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• v.53 no.6
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• pp.694-700
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• 2010

Purpose: To assess the validity of individual and combined prognostic effects of severe bronchopulmonary dysplasia (BPD), brain injury, retinopathy of prematurity (ROP), and parenteral nutrition associated cholestasis(PNAC). Methods: We retrospectively analyzed the medical records of 80 extremely low birthweight (ELBW) infants admitted to the neonatal intensive care unit (NICU) of the Severance Children's Hospital, and who survived to a postmenstrual age of 36 weeks. We analyzed the relationship between 4 neonatal morbidities (severe BPD, severe brain injury, severe ROP, and severe PNAC) and poor outcome. Poor outcome indicated death after a postmenstrual age of 36 weeks or survival with neurosensory impairment (cerebral palsy, delayed development, hearing loss, or blindness) between 18 and 24 months of corrected age. Results: Each neonatal morbidity correlated with poor outcome on univariate analysis. Multiple logistic regression analysis revealed that the odds ratios (OR) were 4.9 (95% confidence interval [CI], 1.0-22.6; $P$=0.044) for severe BPD, 13.2 (3.0-57.3; $P$<.001) for severe brain injury, 5.3 (1.6-18.1; $P$=0.007) for severe ROP, and 3.4 (0.5-22.7; $P$=0.215) for severe PNAC. Severe BPD, brain injury, and ROP were significantly correlated with poor outcome, but not severe PNAC. By increasing the morbidity count, the rate of poor outcome was significantly increased (OR 5.2; 95% CI, 2.2-11.9; $P$<.001). In infants free of the above-mentioned morbidities, the rate of poor outcome was 9%, while the corresponding rates in infants with 1, 2, and more than 3 neonatal morbidities were 46%, 69%, and 100%, respectively. Conclusion: In ELBW infants 3 common neonatal mornidifies, severe BPD, brain injury and ROP, strongly predicts the risk of poor outcome.

• Advances in pediatric surgery
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• v.2 no.1
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• pp.17-25
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• 1996

Acute pancreatitis(AP) in children is not common but can be associated with severe morbidity rates and its diagnosis is often delayed. Thus, reported mortality rates range from 0 to 78%. We have treated 26 patients with AP from 5 to 17 years of age over the past 17 years. We are intended to assess the relevance of the prognostic criteria used to assess severity of adult AP and to review the etiology, clilical presentation, diagnosis, and management of AP in children. The authors retrospectively reviewed 26 children with AP managed in Kyung Hee University Hospital from 1978 to 1995. Among 26 patients with AP, male were 12, and female were 14. And the mean age of patients was 11.8 years. In 9(34.6%), no definitive cause was identified. Common causes of AP were trauma(23.1%) and biliary tract disease(23.1%). Other etiologies were viral infection(15.4%) and post ERCP(3.8%). The presenting features were abdominal pain(92.3%), vomiting(61.5%), fever(19.2%), submandibular pain(11.5%), and abdominal mass(7.6%). Back pain was rare(3.8%). Abdominal ultrasonographic findings were abnormal in 10 of 16 patients(62.5%) and abdominal CT findings were abnormal for 9 of 9 patients(100%). Seventeen patients(65.3%) were managed conservatively, and nine patients(34.6%) required surgical treatment. There was no mortality. To evaluate the severity of disease, we used the Imrie prognostic criteria used to assess the severity in adult AP. The number of positive criteria was correlated to the duration of hospitalization(r2=0.91) but statistically insignificant(p>0.05). But, the number of positive criteria was correlated to the operative incidence(r2=0.93) and statistically significant(p<0.05). The common causes of AP in children were unknown origin(34.6%), trauma(23.1%), and biliary tract disease(23.1%). Ultrasonography and computed tomography were useful imaging tools of AP in children. The Imrie criteria used to evaluate the severity in adult AP were suspected to be valuable to assess the severity of AP in children.

• Clinical and Experimental Pediatrics
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• v.63 no.11
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• pp.438-446
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• 2020

Background: Most developmental screening tools in Korea are adopted from foreign tests. To ensure efficient screening of infants and children in Korea, a nationwide screening tool with high reliability and validity is needed. Purpose: This study aimed to independently develop, standardize, and validate the Korean Developmental Screening Test for Infants and Children (K-DST) for screening infants and children for neurodevelopmental disorders in Korea. Methods: The standardization and validation conducted in 2012-2014 of 3,284 subjects (4-71 months of age) resulted in the first edition of the K-DST. The restandardization and revalidation performed in 2015-2016 of 3.06 million attendees of the National Health Screening Program for Infants and Children resulted in the revised K-DST. We analyzed inter-item consistency and test-retest reliability for the reliability analysis. Regarding the validation of K-DST, we examined the construct validity, sensitivity and specificity, receiver operating characteristic curve analysis, and a criterion-related validity analysis. Results: We ultimately selected 8 questions in 6 developmental domains. For most age groups and each domain, internal consistency was 0.73-0.93 and test-retest reliability was 0.77-0.88. The revised K-DST had high discriminatory ability with a sensitivity of 0.833 and specificity of 0.979. The test supported construct validity by distinguishing between normal and neurodevelopmentally delayed groups. The language and cognition domain of the revised K-DST was highly correlated with the K-Bayley Scales of Infant Development-II's Mental Age Quotient (r=0.766, 0.739), while the gross and fine motor domains were highly correlated with Motor Age Quotient (r=0.695, 0.668), respectively. The Verbal Intelligence Quotient of Korean Wechsler Preschool and Primary Scales of Intelligence was highly correlated with the K-DST cognition and language domains (r=0.701, 0.770), as was the performance intelligence quotient with the fine motor domain (r=0.700). Conclusion: The K-DST is reliable and valid, suggesting its good potential as an effective screening tool for infants and children with neurodevelopmental disorders in Korea.

• Phonetics and Speech Sciences
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• v.13 no.2
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• pp.91-101
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• 2021

The purpose of this study was to investigate laryngeal characteristics in children with autism spectrum disorders (ASD). A total of 50 children participated, including eight children aged 2 to 4 years old diagnosed with ASD and 42 normal controls at the same age. All children recorded X-ray images of the midsagittal plane of the cervical spine and larynx, and compared the laryngeal positions of ASD and control. In addition, samples of children with vowel prolongation were collected and analyzed for acoustic parameters. X-rays showed that the height of the hyoid bone in the normal group was the lowest at 3 years of age, and ascended at 4 years of age. Nevertheless, the distance from the external acoustic meatus to the hyoid bone was longest at age 4. 4-year-olds with explosive language development showed laryngeal height elevation and anteriorization. In contrast, the hyoid height of the ASD group of all ages was lower than that of the control group, and there was no difference in the hyoid position between the ages. As a result of acoustic evaluation, PFR, vFo, and vAm were significantly higher ASD than control. Low laryngeal height of ASD children may be associated with delayed language development. PFR, vFo, and vAm seem to be voice markers showing the difference between normal and ASD children.