• Childhood Kidney Diseases
• /
• v.13 no.2
• /
• pp.161-169
• /
• 2009

Purpose : This study was performed to report the diagnosis and treatment of nephrotic syndrome manifesting in the first year of life. Methods : We retrospectively reviewed the clinical data with chart review in 7 patients who were diagnosed as nephrotic syndrome manifesting in the first year of life from 1996 to 2007. Results : Three patients had congenital nephrotic syndrome, the other 4 patients had infantile nephrotic syndrome. Their ages ranged from birth to 11 months and male to female ratio was 1 to 6. Renal biopsies were done in 6 patients. One patient had Finnish type congenital nephrotic syndrome, 2 patients had diffuse mesangial sclerosis, 2 patients had focal segmental glomerulosclerosis and 1 patient had minimal change disease. Genetic analyses of NPHS2, PLCE1, and WT1 were done in 4 patients and 2 of them had WT1 mutation. Among 3 patients with congenital nephrotic syndrome, 1 patient was diagnosed as congenital nephrotic syndrome of Finnish type and the other 2 patients were diagnosed as Denys-Drash syndrome. All of the patients with congenital nephrotic syndrome died due to sepsis. Among 4 patients with infantile nephrotic syndrome, 2 patients died and 1 had remission, another patient progressed to end stage renal disease. Conclusion : Most of nephrotic syndrome manifesting in the first year was hereditary renal disease. Patients with nephrotic syndrome manifesting in the 3 month of life had poorer prognosis and needed more aggressive management including early dialysis and renal transplantation might be considered compared with infantile nephrotic syndrome. Further genotype-phenotype correlation studies are needed.

• Childhood Kidney Diseases
• /
• v.13 no.2
• /
• pp.130-137
• /
• 2009

Purpose : Stem cell transplantation (SCT) has gained worldwide acceptance as a treatment for hematologic disorders. This study was performed to evaluate the clinical characteristics and outcomes of the acute kidney injury after SCT in children. Methods : The records of 53 patients who were treated with SCT at the pediatric department of Yeungnam University Hospital between January, 1996 and April, 2009 were used as subjects. Their were divided into two groups ; 'Early renal insufficiency' (ERI, n=18) and 'Non-early renal insufficiency' (NERI, n=35). ERI had greater than 25% of drop in GFR after SCT. Results: Total 53 patients were analyzed. In cord blood SCT (n=11), ERI was 4 (36.4%) and NERI was 7 (63.6%). In bone marrow SCT (n=16), ERI was 8 (50.0%) and NERI was 8 (50.5%). In autologous peripheral blood SCT (n=26), ERI was 6 (23.1%) and NERI was 20 (76.9%). There is no difference in both groups according to kinds of SCT. GVHD was developed in 22 patients, and there is no difference in each group. Twenty two of 53 patients died. ERI was 12 (66.7%) and NERI was 10 (28.6%). Acute renal failure is most important cause of the deaths. Conclusion : Out of 53 pediatric patients who were treated with SCT, 18 patients had greater than 25% of drop in GFR. There is no difference in both groups according to kinds of SCT. GVHD was found in 22 patients and there is no relation between GVHD development and acute kideney injury.

• Journal of Chest Surgery
• /
• v.42 no.2
• /
• pp.175-183
• /
• 2009

Background: Mitral valve abnormalities in the pediatric population are rare. Mitral valve replacement or pediatric mitral lesions can cause problems such as a lack of growth potential. There re only limited experiences with mitral valve repair at any institution, so the purpose of his study is to evaluate the outcomes of mitral valve repair n pediatric patients. Material and Method: Sixty-four consecutive children (28 males and 36 females) with a mean age of $5.5{\pm}4.7$ years underwent mitral valve repair for treating their congenital mitral valve disease between January 1996 and December 2005. The patients were divided into two groups: group 1 (34 patients (53.1%)) had isolated disease (mitral anomaly with or without trial septal defect or patent ductus arteriosus) and group 2 (30 patients (46.9%)) had complex disease (mitral anomaly with concurrent intracardiac disease, except atrioventricular septal defect). Result: The overall in-hospital mortality was 6.3%; group 1 had 5.9% mortality and group 2 had 10.0% mortality. The postoperative morbidity was 18.8%; group 1 and 2 had 14.7% and 23.3% postoperative morbidity, respectively, and there as no significant difference among the groups. The median follow-up was 4.6 years range: $0.5{\sim}12.2$ years). The 10-year survival rate was 95.3%. The 10-year freedom from re-operation rate was 76.1% with 10 re-operations. The majority of the functional classifications were annular dilatation and leaflet prolapse. A mean of $2.1{\pm}1.1$ procedures per patient were performed. The echocardiography that was done at the immediate postoperative period showed a significant improvement in the mitral valve function. The follow-up echocardiographic results were significantly improved. However, mitral stenosis newly developed over time, and there ere significant differences according to the repair strategies. Conclusion: The patients who underwent mitral valve repair for congenital mitral anomalies showed good results. The follow-up echocardiography revealed satisfactory short-term and long-term results. Close follow-up is necessary to detect the development of postoperative mitral stenosis or regurgitation.

• Journal of Yeungnam Medical Science
• /
• v.13 no.2
• /
• pp.211-224
• /
• 1996

The patients of developmental dislocation of the hip(DDH) are almost found after walking age because of early diagnosis of DDH in younger children is not easy. A controversy still exists as to the relative value of closed and operative management in the treatment of a child who has reached walking age. This study is a report of the results of 16 patients(17 cases) in developmental dislocation of the hip who have visited our hospital at the age of 9 months to 3 years old, and have been followed more than 12 months (12-112 months) on review of plain radiographs and arthrograms. The results were as follows : 1. The age at diagnosis was 16.4 months(9-31 months) in average. The methods of treatment were conservative for 8 cases, and operation for 4 cases and secondary operation for 5 cases who were failed with conservative therapy. 2. By Severin's radiologic grade, the result was good in 4 cases, fair in 3 cases and poor in 1 case in conservative treatment. In operative treatment, fair was in 2 cases and poor in 2 cases. In secondary operative patients who were failed with conservative therapy, good was in 2 cases, fair in 2 cases and poor in 1 case. 3. Avascular necrosis of femoral head was developed in 3 cases. 4. In the good reduction as determined by arthrogram according to Race and Herring, we can get a favorable result in conservative treatment, and in the poor reduction as determined by arthrogram, the better result in operative treatment can be expected.

• Clinical and Experimental Pediatrics
• /
• v.49 no.11
• /
• pp.1158-1166
• /
• 2006

Purpose : The purpose of this study is to make a diagnostic classification and discuss a diagnostic strategy of floppy infants by investigating clinical, neurological, electrophysiological, and genetic analysis of infants admitted to intensive care units with the complaint of hypotonia. Methods : A retrospective study was performed from Jan. 1993 to Dec. 2005 in neonatal and pediatric intensive care units of Seoul National University Children's Hospital. Clinical features and all tests related to hypotonia were investigated. Results : There were 21 cases of floppy infants admitted to intensive care units. Final diagnosis was classified as centra (7 cases[33.3 percent]), peripheral (11 cases [52.4 percent]), and unspecified (3 cases [14.3 percent]). Among the central group, three patients were diagnosed as hypoxic ischemic encephalopathy, two patients as Prader-Willi syndrome, one patient as chromosomal disorder, and one patient as transient hypotonia. Among the peripheral group, four patients were diagnosed as myotubular myopathy, three patients as SMA type 1, two patients as congenital myotonic dystrophy, one patient as congenital muscular dystrophy, and one as unspecified motor-neuron disease. Motor power was above grade 3 on average, and deep tendon reflex was brisk in the central group. Among investigations, electromyography showed 66 percent sensitivity in the peripheral group, and muscle biopsy was all diagnostic in the peripheral group. Brain image was diagnostic in the central group, and Prader-Willi FISH or karyotyping was helpful in diagnosis in central group. Morbidity and mortality was more severe in the peripheral group Conclusion : Classification of diagnosis by clinical characteristics in this study, and application of investigations step by step, may provide an effective diagnostic strategy.

• Clinical and Experimental Pediatrics
• /
• v.49 no.11
• /
• pp.1211-1215
• /
• 2006

Purpose : In this study, we analyzed the short term changes of thyroid function, incidence and risk factors of thyroid dysfunction soon after allogeneic hematopoietic stem cell transplantation (HSCT) in children. Methods : We enrolled 80 pediatric patients following allogeneic HSCT, at the Catholic HSCT center between January, 2004 and February, 2006. Serum TSH (thyroid stimulating hormone), total serum thyroxine and total serum triiodothyronine levels were systematically measured in 80 patients before the HSCT, and at 1 month, 6 months and 12 months after HSCT. Results : Thyroid function statistically decreased at 1 month after HSCT(P<0.001). Thyroid dysfunction at 1 month was observed in 43 (54 percent) of 80 patients, 31 (39 percent) of whom presented with euthyroid sick syndrome (ETS). Thyroid dysfunction was normalized within 1 year after HSCT. In univariate analysis, malignant disease and the presence of acute graft-versus-host disease (grade ${\geq}II$) were risk factors for ETS (P=0.04, 0.01 respectively). In multivariate analysis, we could not detect an independent risk factor for ETS (P=0.19, 0.06 respectively). Conclusion : The present study suggests that the incidence of thyroid dysfunction is high after allogeneic HSCT. Therefore, regular monitoring of thyroid hormone levels after HSCT is required.

• Clinical and Experimental Pediatrics
• /
• v.45 no.4
• /
• pp.505-511
• /
• 2002

Purpose : Mean platelet volume(MPV) and platelet distribution width(PDW) are useful parameters in evaluating disorders of platelets. In cases with idiopathic thrombocytopenic purpura(ITP), they change as platelet count increases. In this study, we compared the values of MPV and PDW in ITP patients at diagnosis with those of normal children. We also studied whether the early changes in MPV may predict the clinical course. Methods : From December 1995 to May 2001, 71 patients with ITP were admitted to Ajou University Hospital. They were treated with IVIg 400 mg/kg for five days and MPV, PDW, platelet count were analysed. Normal control group(n=38) was compared. The study group was divided into acute and chronic forms, and also divided into group A, good early responders whose platelets increased more than $100,000/{\mu}L$ within 5 days and group B who did not. Results : Mean value of MPV at diagnosis in ITP patients was lower than the normal control group(P<0.05). In group A, MPV was abruptly increased on the first day after IVIg and then started to decrease. But in group B, MPV was steadily increased until the fourth day after IVIg. In the normal control group, there were inverse correlations between platelet count and MPV(r=-0.415, P<0.05), but in ITP patients, there were positive relationships between platelet count and MPV(r=0.646, P<0.05) at diagnosis. Conclusions : MPV at diagnosis of ITP was lower than the normal control. MPV and PDW could not predict the course of ITP patients, but MPV could distinguish good early responders. More research is needed to find out the reasons of decreased MPV at diagnosis of ITP.

• Clinical and Experimental Pediatrics
• /
• v.45 no.2
• /
• pp.192-198
• /
• 2002

Purpose : It is important to select and use kinds of Inhaler appropriate to the age of the patient and severity of symptoms. Several kinds of Inhaler have been developed and recommended according to each inhaler's resistance and usage method. We evaluated the usable age of 4 inhalers (turbulent flow inhalers, multi unit dose inhalers, breath actuated MDI, and autohalers) by measuring peak inspiratory flow(PIF) using $In-Check^{(TM)}$ Inspiratory Flow Meter. Methods : Ninety three patients aged from 3 to 7 years(mean $57{\pm}12.9$ mo.) who had admitted to CHA hospital from July 2000 to April 2001 were enrolled. Study patients were divided into 4 groups according to age : 3-4 years of age(group A, n=31), 4-5 years of age(group B, n=32), 5-6 years of age(group C, n=18), and 6-7 years of age(group D, n=12). Results : Out of total 93 patients, 23(71%), 27(84%), 17(94%) patients of each group A, B, C and all 12 patients of group D could use the 4 types of inhaler through adequated education. In all four groups, height, body weight and age were significantly correlated(P<0.05). Usable age, height and weight of children who can use turbulent flow inhaler(TFI) were each 8 year 7 month old, 144 cm, 32.0 kg, and those of multi unit dose inhaler(MUD) were 2 year 10 month old, 92 cm, 12.0 kg. Also that of breath-actuated MDI were 1 yr 8 months, 83 cm, 8.5 kg and that of autohaler were 2 yr 8 months, 91 cm, 11.0 kg Conclusion : We concluded that the ability to use inhalers correlated with height, weight and age of the patients. Multi unit dose inhalers, Breath actuated MDI and Autohaler are useful after 3 years of age through adequate education.

• Clinical and Experimental Pediatrics
• /
• v.51 no.4
• /
• pp.383-390
• /
• 2008

Purpose : This study was aimed to analyze the clinical characteristics of patients with acute interstitial pneumonia who had presented similar clinical patterns from March to June, 2006 and to describe our experience of treatment and to identify risk factors associated with prognosis. Methods : The clinical characteristics, radiologic and histopathologic findings and response to steroids of 15 patients (non-survival group [n=7] and survival group [control, n=8]) with acute interstitial pneumonia were investigated through the review of medical records. Results : The mean age of the patients was 26 (range: 3-48) months. Cough, cyanosis and fever were frequent symptoms. The most frequent radiologic findings on admission were pneumomediastium and extensive ground glass opacity. Surgical lung biopsy was performed on 8/15 (53.3%) patients and diffuse alveolar damage was found. Mechanical ventilation was applied for 9/15 (60.0%) patients for 40 (range: 1-99) days. Five patients in survival group received steroid treatment and 7 patients in non-survivial group (P=0.20). One patient in survival group received steroid pulse treatment and 4 patients in non-survival group (P=0.12). Seven patients died all of respiratory failure. The survival rate was 53.4%. Conclusion : The patients with acute interstitial pneumonia which occurred on spring 2006 showed high mortality because of rapidly and extensively progressing pulmonary fibrosis and air leakage. Therefore, we should consider surgical lung biopsy and steroid application earlier. We should recognize this acute interstitial pneumonia occurring on spring in domestics and need to investigate the cause and treatment in large scale.

• The Korean Nurse
• /
• v.34 no.3
• /
• pp.85-98
• /
• 1995

The purpose of this study is to provide basic data regarding effective learning opportunities in childbirth education classes. Also analysis of the data indicates the optimum conditions for the welfare and improvements in the promotion of health in childbearing mothers. The results of this study are as follows; 1) The average age of the subjects in this study was 30.6 years and the total number of subjects was 58 pregnant women. The average number of children was one and 84.5% of the subjects were unemployed even though 63.8% of them held over bachelor's degrees. It was found that 22.4% of the subjects were living in an extended family. Also 61.5% of them were living with parents-in-law. The number of pregnancies were calssified as one, two, or three to nine times with the percentages of 58.7%, 22.4% and 18.9%, respectively. Further, 72.4% of the subjects had no abortion experience and 15.5% had one aborion experience. While 89.7% of the subjects planned to feed their babies with breastmilk, mixed feeding were used by only 22.4% of the sample. These data were collected at about 6 months after delivery. Thus one can see that a low rate of breastfeeding was common. 2) The length of one period of childbirth education is four weeks. It was found that 36.2% of the subjects participated in childbirth education only once, where as 13.8% participated four times and 19% of the subjects participated in this class more than four times. pregnant at least once. Further, 75.9% of the participants were participated in this education through their own will. Their motivation for participation developed through information, advertisement and posters which contained information on childbirth education. Those with unplanned pregnancies 92.9% participated after a suggestion by the nurses. The number of participants in terms of percentage according to the childbirth education contents can be classified as following. The most active participation was shown in preparation of delivery(77.6%), postpartrm management(56.9%) fetal development(37.6%) and physiology of pregnancy(17.2%). It was found that 75.9% of the subjects were willing to participate again if they were given a chance. The reason can be summarized as following: The content of the education is very helpful(47.7%). Scientific knowledge can be obtained through this program(20.5%). Participation helps in achieving psychological stability(9.1%). Participation enables one to establish a friendly relationship with other participants(6.8%) of the sample. 24.1% of the participants did not want to participate again. The reasons can be as following: They do not want another baby(42.9%). The first paricipation in childbirth education gave enough knowledge about childbirth(21.4%). Another reason for not want to participate again was because they had a cesarean birth(14.3%). Only 7.1% of them responded with a negative view. A response that they do not need childbirth education after their operation can be traced back to the general belief that childbirth education is the place where one prepares for natural birth through the Lamaze breathing technique. Of the subjects, 91.4% suggested that this program could be recommended to other childbearing mothers, because this program gave educational content along with psychological stability for childbearing women. Of the subjects 41.4% did not see any efforts towards the welfare of the baby, where as 88.2% did. Among the subjects 58.6% made some effort to eliminate the discomfort of labor by breathing and imagination and breathing and walking. Further 41.7% of the 24 subjects did not do anything toward the welfare of the baby, because they did have a cesarean section so that they didn't have a chance even though they had been educated about childbirth. Also 33.3% of the subjects did not do anything toward the welfare of the baby, because they lacked a willingness. After leaving the hospital, only 75.9% of the subjects did some exercises. The subjects who tried participate this program with their husband accounted for 20.7% of the sample. Interviewing with the subjects solved some of the uneasiness and. fear of delivery, increased self-confidence in parenting and active coping in the delivery process.