• Clinical and Experimental Pediatrics
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• v.49 no.3
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• pp.251-257
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• 2006

Purpose : In this study, we created a questionnaire and collected answers concerning actual conditions of vaccination and parents' knowledge of vaccination issues, in order to find false knowledge of patients. We tried to give correct information and suggested the role of the pediatrician. Methods : We made questionnaires and collected answers from 466 parents from March 2004 to June 2004. Results : Places of vaccination were pediatric clinics(49.4 percent), health centers(27.7 percent), general/university hospitals(15.0 percent) and other clinics(4.9 percent). We found 38.8 percent of parents thought that the reason for vaccination at pediatric clinics was a belief of speciality, even though there is no difference in the vaccination itself. We also found 15.0 percent of parents thought that there were no differences between pediatricians and other physicians, but 52.0 percent of parents wanted to receive vaccination at pediatric clinics in the future. Our study also found that 62.4 percent of parents wanted to make out a preliminary questionnaire for vaccination. Many parents got vaccination information from vaccination record books(57.9 percent), and 52.6 percent of parents incorrectly believed that Hib vaccination could prevent all kinds of meningitis. Conclusion : Our study suggests that pediatricians need to make efforts to give out correct information. It is necessary to use preliminary questionnaires for vaccination and correct vaccination information should be written on the vaccination record book. Also, there needs to be counselling with parents about weaning, growth and development at the time of vaccination, and to point out the differences between pediatricians and other physicians.

• Clinical and Experimental Pediatrics
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• v.51 no.8
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• pp.812-819
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• 2008

Purpose : Vitamin D plays a key role in bone mineralization of the skeleton and vitamin D deficiency can lead to rickets. It is well known that vitamin D deficiency is common in breast fed infants. Of these patients, clinically, some have no signs of rickets, but laboratory and radiographic findings are diagnostic for vitamin D deficiency rickets (subclinical vitamin D deficiency rickets). The purpose of this study is to clarify current causes and ways to prevent this disease. Methods : We reviewed the clinical and laboratory characteristics of children who were incidentally diagnosed as subclinical rickets during treatment of other disease such as pneumonia, gastroenteritis, urinary tract infection at Eulji Hospital, Seoul, Korea from March, 2003 to July 2007. Results : Eight patients (six boys and two girls) were diagnosed with subclinical vitamin D deficiency rickets. The mean age of the patients was $12.6{\pm}5.8months$, and they were diagnosed from January to July. The associated diseases were pneumonia, urinary tract infection, acute gastroenteritis, and iron deficiency anemia. All patients were breast-fed. Two showed growth failure. The mean serum alkaline phosphatase was $1995.8{\pm}739.5IU/L$, the mean calcium count was $9.5{\pm}0.6mg/dL$, and the mean phosphorus content was $3.6{\pm}1.5mg/dL$. The mean intact parathyroid hormone was $214.8{\pm}155.9pg/mL$ (reference range, 9-65), the mean 1,25-dihydroxyvitamin D was $82.4{\pm}49.3pg/mL$ (reference range, 2070), and the mean 25-hydroxyvitamin D was $29.6{\pm}10.6ng/mL$ (reference range, 1030). A radiographic examination showed cupping, fraying, and flaring of metaphyses in all patients. Six patients were administered calcitriol (400 IU/day) for three months. A consequent radiographic and laboratory examination showed improvement. The first two patients were initially diagnosed with metaphyseal dysplasia, without the detection of vitamin D deficiency and they spontaneously improved without vitamin D supplements. However, two years later, they showed mild scoliosis and metaphyseal dysplasia, respectively. Conclusion : Breast-feeding without supplementation involves high risk of vitamin D deficiency. Some infants may also develop rickets; therefore, such groups should be considered for vitamin D supplementation.

• Clinical and Experimental Pediatrics
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• v.51 no.8
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• pp.827-833
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• 2008

Purpose : Early identification of iron deficiency in young children is essential to prevent damaging long-term consequences. It is often difficult for the pediatrician to know which indices should be used when diagnosing these conditions especially in hospitalized young children. This study investigated the clinical significances of reticulocyte hemoglobin content in young children with acute infection. Methods : We studied 69 young children aged from 6 to 24 months admitted with acute infection in a single center. Venous blood was drawn to determine hemoglobin (Hb), mean corpuscular volume (MCV), mean corpuscular hemoglobin (MCH), hemoglobin content (CH), reticulocyte hemoglobin content (CHr), and red blood cell distribution width (RDW) using ADVIA 120 (Bayer Diagnostics, NY, USA). For evaluating iron status, iron, total iron binding capacity, ferritin and transferrin saturation (Tfsat) were determined. Iron deficiency was defined as Tfsat less than 20%, and iron deficiency anemia as Tfsat less than 20% and Hb level less than 11 g/dL. Results : In all, 47 were iron deficient; 17 of these had iron deficiency anemia. CHr was the only significant predictor of iron deficiency (likelihood ratio test=71.25; odds ratio=0.67; P<0.05). Plasma ferritin level had no predictive value (P=0.519). Subjects with CHr less than 27.4 pg had lower Hb level, MCH, CH, Tfsat, and iron levels than those with CHr 27.4 pg or more (P<0.05 for all). Conclusion : CHr level was a sensitive screening tool and the strongest predictor of iron deficiency in hospitalized infants with acute infection; it was cost saving and avoiding additional sampling. However its reference range should be established.

• Clinical and Experimental Pediatrics
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• v.45 no.12
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• pp.1503-1511
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• 2002

Purpose : Although the short- and long-term outcomes of low birth weight(LBW) neonatal intensive care unit(NICU) survivors have been extensively studied, much less information is available for normal birth weight(NBW) infants(greater than 2,500 gm) who require NICU care. Methods : We retrospectively examined the neonatal hospitalizations and one year health status of 302 NBW and 131 LBW admissions to our NICU. Information on the neonatal hospitalization was obtained from a review of medical records. Postdischarge health status was collected by using telephone surveys and medical records. Results : After initial discharge, 21.2% of the NBW infants and 23% of the LBW infants required rehospitalization during the first year of life and there was no significant difference between the two groups. The reasons for rehospitalization of the NBW infants included respiratory disorders (32.1%), G-I problems(26.2%), genitourinary problems(11.9%), surgery(10.7%), cardiac problems(7.1%), and congenital/developmental problems(1.2%). For the LBW infants, the order of frequency was the same, with the percentages slightly different. Neonatal risk factors related to the rehospitalization of the NBW infants included mechanical ventilation, duration of mechanical ventilation, and congenital anomaly. But no positive significant correlation of neonatal risk factors with rehospitalization of LBW infants was found. Conclusion : Low and normal birthweight NICU survivors were rehospitalized at similar rates. The most common cause of rehospitalization was respiratory problems. Neonatal risk factors related to rehospitalization of NBW infants were mechanical ventilation, duration of mechanical ventilation, and congenital anomaly. However, no positive significant correlation of neonatal risk factors with rehospitalization of LBW infants was found. The data suggests that NBW infant survivors, as well as LBW infant NICU survivors, require close follow up.

• Clinical and Experimental Pediatrics
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• v.45 no.11
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• pp.1403-1410
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• 2002

Purpose : In previous studies, various risk factors for recurrent febrile seizures have been identified. But none of these risk factors alone could sufficiently discriminate children at high or low risk for recurrent seizures. Therefore, we tried to identify patients at high risk of recurrent febrile seizures by combining risk factors. Methods : Two hundred and four children who had been admitted to our hospital from March, 1997 to July, 1999 with their first febrile seizures were enrolled in our study, and followed up over 2 years. We investigated the recurrence rate according to variables such as sex, age at first febrile seizure, family history of febrile seizures or epilepsy, type of the first seizure, neurologic abnormality and EEG abnormality. Results : Family history of febrile seizures and age at first febrile seizure(<12 months) were significant independent risk factors for recurrent febrile seizures. With these two combined factors, four groups were allocated and the recurrence rate by each group was designated as follows: group with no family history of febrile seizures and age at first febrile seizure ${\geq}12$ months (no risk factor), 43.8%; the group with no family history and age <12 months(one risk factor), 61.7%; group with family history and age ${\geq}12$ months(one risk factor), 64.5%; group with family history and age <12 months(two risk factors), 90.4%. Conclusion : A correlation between numbers of risk factors and recurrence rate was present and the children with a family history of febrile seizures and a young age at onset(<12 months) were regarded as a high risk group of recurrence.

• Clinical and Experimental Pediatrics
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• v.45 no.12
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• pp.1551-1558
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• 2002

Purpose : Loss of hippocampal interneurons in dentate gyrus has been reported in patients with severe temporal lobe epilepsy and in animals treated with kainic acid(KA). Interneurons contain $Ca^{2+}$- binding protein parvalbumin(PV). The effects of kainic acid on parvalbumin-immunoreactive (PV-IR) interneurons in dentate gyrus were investigated in organotypic hippocampal slice cultures. Methods : Cultured hippocampal slices from postnatal day nine C57/BL6 mice were exposed to $10{\mu}M$ KA, and were observed at 0, 8, 24, 48, 72 hours after a one hour KA exposure. Neuronal injury was determined by morphologic changes of PV-IR interneuron in dentate gyrus. Results : Transient(1 hour) exposure of hippocampal explant cultures to KA produced marked varicosities in dendrites of PV-IR interneuron in dentate gyrus and the shaft of interbeaded dendrite is often much thinner than those in control. The presence of varicosities in dendrites was reversible with KA washout. The dendrites of KA treated explants were no longer beaded at 8, 24, 48 and 72 hours after KA exposure. The number of cells in PV-IR interneurons in dentate gyrus was decreased at 0, 8 hours after exposure. But there was no significant difference in 24, 48 and 72 hours recovery group compared with control group. Conclusion : The results suggested that loss of PV-IR interneurons in dentate gyrus is transient, and is not accompanied by PV-IR interneuronal cell death.

• Clinical and Experimental Pediatrics
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• v.53 no.4
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• pp.532-537
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• 2010

Purpose : Early postoperative arrhythmias are a major cause of mortality and morbidity after open heart surgery in the pediatric population. We evaluated the incidence and risk factors of early postoperative arrhythmias after surgery of congenital heart disease. Methods : From January 2002 to December 2008, we retrospectively reviewed the medical records of the 561 patients who underwent cardiac surgery in Kyungpook National University Hospital. We analyzed patients' age and weight, occurrence and type of arrhythmia, cardiopulmonary bypass (CPB) time, aortic cross clamp (ACC) time, and postoperative electrolyte levels. Results : Arrhythmias occurred in 42 of 578 (7.3%) cases of the pediatric cardiac surgery. The most common types of arrhythmia were junctional ectopic tachycardia (JET) and accelerated idioventricular rhythm (AIVR), which occurred in 17 and 13 cases, respectively. The arterial switch operation (ASO) of transposition of the great arteries (TGA) had the highest incidence of arrhythmia (36.4%). Most cases of cardiac arrhythmia showed good response to management. Patients with early postoperative arrhythmias had significantly lower body weight, younger age, and prolonged CPB and ACC times ($P$<0.05) than patients without arrhythmia. Although the mean duration of ventilator care and intensive care unit stay were significantly longer ($P$<0.05), the mortality rate was not significantly different among the 2 groups. Conclusion : Early postoperative arrhythmias are a major complication after pediatric cardiac surgery; however, aggressive and immediate management can reduce mortality and morbidity.

• Clinical and Experimental Pediatrics
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• v.50 no.2
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• pp.182-189
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• 2007

Purpose : The primary purpose of this study was to evaluate the growth and neuropsychologic function following treatments for pediatric hematologic and oncologic diseases. Healthy monozygotic twins served as ideal controls for comparison to exclude possible confounding factors. Methods : Seven children treated with various hematologic and oncologic diseases were included in the study: acute lymphoblastic leukemia (ALL; n=2), Diamond-Blackfan anemia twins (n=2), and aplastic anemia (n=3). The median age at the diagnosis was 5.2 (0.3-15) years. The median duration of follow-up was 7.2 (4.9-10) years. Controls were healthy monozygotic twins. Growth was measured and the percentile channels were evaluated sequentially for patients. The K-WISC III was applied and compared in 5 pairs of patients and controls. Results : Similar growth profiles were noted for the twins. The percentiles at diagnosis was 3-10 in 3, 25-50 in 2, and 50-75 in 2 cases. All patients stayed in their growth percentiles through follow-up, except for 1 patient who became obese. For IQ tests, the mean behavioral, verbal and full scale IQ scores of patients were 88.0, 93.8, and 89.8, respectively, and those from their corresponding controls were 92.2, 97.0, and 91.7 (P>0.05). However, 2 children who were treated for ALL had lower IQ scores. Conclusion : Similar growth profiles were observed in the monozygotic twins in terms of height and weight. The IQ scores of patients were similar to those of monozygotic twins. However, prophylactic CNS-directed therapy for leukemia might adversely affect the IQ scores. A further prospective study on larger number of twins is warranted.

• Clinical and Experimental Pediatrics
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• v.51 no.11
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• pp.1172-1178
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• 2008

Purpose : Congenital chylothorax is an accumulation of lymphatic fluid within the pleural space. It is a common cause of unidentified hydrops fetalis. We examined the perinatal history, clinical manifestation, diagnosis, treatment, and outcome in 6 newborns diagnosed to have congenital chylothorax with hydrops fetalis. We also studied the effect of octreotide therapy for congenital chylothorax in relation to conservative treatment. Methods : We retrospectively reviewed the medical records of 6 patients diagnosed to have congenital chylothorax with hydrops fetalis among 27,907 newborns who were born at the Cheil General Hospital and Womens Healthcare Center between January 2004 and July 2007. The diagnosis of chylothorax is based on the analysis of pleural fluid before and after milk feeding. Results : Incidence of congenital chylothorax in this study was 0.021%. All 6 cases were noted in over the 92% lymphocyte in pleural analysis. Transudate was changed into chyle with increasing triglyceride levels above 200 mg/dL after milk feeding. Three of 6 infants improved with conservative treatment, including thoracostomy and assisted ventilation. The others had persistent symptoms despite conservative treatment and responded to octreotide therapy. A complication, specifically vomiting was noted in 1 case during octreotide therapy. Conclusion : In this study, octreotide therapy resulted in a safe and excellent outcome. Therefore, octreotide therapy is considered in severe refractory congenital chylothorax in conservative treatment. Further studies are required to determine appropriate guidelines for octreotide therapy.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.14 no.2
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• pp.142-149
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• 2014

Purpose: There are 15 types of Glycogen storage disease (GSD) that have been identified, and GSD type Ia is the most common type. There are several studies of Korean GSD type Ia patients' long-term complications. The aim of this study to find out clinical symptoms and prognosis of GSD type Ia patients. Methods: We performed clinical, biochemical and genetic analysis retrospectively on five patients diagnosed with GSD type Ia in a Soonchunhyang University Hospital from July 2002 to July 2014. Results: All patients had hepatomegaly at diagnosis, and they were all confirmed to have fatty liver at abdomen USG. They had no developmental delay, but two of them had growth retardation. Elevated blood lactate, triglyceride, and uric acid levels can find out all patients, but only one patient had hypoglycemia. They are diagnosed with GSD through gene analysis, and by gene analysis, they have c.648G>T (homozygote, splicing mutation), c.122G>A/c.648G>T, c.248G>A/c.648G>T mutations. Treatment with three times meals, three times snacks and four to six times use of uncooked constarch for all patients. Following the progress, one of them resulted in hypothyroidism, other one had renal stones. A patient diagnosed at 16 years old had liver cirrhosis and started having hemodialysis for ESRD. Conclusion: GSD type Ia patients had hepatomegaly, hyperlipidemia, hyperuricemia, and lactacidemia. Therefore patients who have such these symptoms are recommended gene analysis. A patient diagnosed at 16-years-old had liver cirrhosis and ESRD in progress, early diagnosis and treatment are important for GSD type Ia patients.