• Clinical and Experimental Pediatrics
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• v.53 no.1
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• pp.56-66
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• 2010

Purpose : Although oseltamivir is widely used for treatment of influenza, few clinical studies of its efficacy and resistance have been performed in Korea. We evaluated the safety, side effects, and efficacy of oseltamivir treatment in Korean pediatric patients. Methods : We analyzed 321 children diagnosed with influenza at Busan St. Mary's Medical Center, Korea, between January 2008 and June 2008 (first study period) and November 2008 and January 2009 (second study period). Patients were divided into two groups: those receiving oseltamivir treatment for 5 days and those receiving only symptomatic treatment. We investigated clinical symptoms, side effects, and resistance to oseltamivir. We also identified influenza strains and evaluated resistance to oseltamivir using an influenza virus culture. Results : One hundred eighty-six patients were assigned to the treatment group, and 135 were assigned to the control group. The treatment group showed shorter admission duration (4.4 days) compared with controls (5.0 days) (P =0.000) and had fewer lower respiratory tract complications compared with controls (P <0.05). No significant statistical difference in the virus antigenic type was observed between the groups. In the first study period, virus culture showed influenza B (41.7% vs. 49.6%), A/H3N2 (7.9% vs. 8.4%), and A/H1N1 (9.4% vs. 6.5%). In the second study period, only A/H1N1 (55.3% vs. 50.0%) was isolated, except for one case of A (H3N2) in the treatment group. No differences in short- and long-term side effects, including neuropsychologic side effects, were noted between groups. There was no resistance to oseltamivir before or after treatment in the first study period. Conclusion : Based on our results, we suggest that osetalmivir therapy in pediatric patients is effective.

• Clinical and Experimental Pediatrics
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• v.52 no.1
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• pp.93-98
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• 2009

Purpose : The purpose of this study was to evaluate the clinical characteristics and outcomes of patients with neuroblastoma aged less than 1 year. Methods : From January 1997 to December 2007, 41 patients aged less than 1 year were diagnosed with neuroblastoma. Patients were divided into 3 risk groups according to the stage of the disease and N-myc amplification. Low-risk patients underwent surgery with (stage 2) or without (stage 1) short-term chemotherapy. Intermediate-risk patients underwent chemotherapy and surgery with or without local radiation therapy. High-risk patients underwent chemotherapy, surgery, radiation therapy, and high-dose chemotherapy/autologous stem cell rescue (HDCT/ASCR). Results : While tumor relapse occurred in only 1 patient, 7 patients died of treatment-related toxicities. Causes of treatment- related death included infection during conventional chemotherapy in 5 patients and acute myocarditis during HDCT/ASCR in 2 patients. The overall 5-year survival (${\pm}$ standard error) and 5-year event-free survival (EFS) rates after diagnosis for all 41 patients were $82.8{\pm}5.9%$ and $80.0{\pm}$6.3%$, respectively, with a median follow-up of 58 (9-137) months. The 5-year EFS rates for low-risk, intermediate-risk, and high-risk patients were 100%, $68.4{\pm}10.8%$, and $66.7{\pm}19.3%$, respectively. Conclusion : Increased efforts to reduce infection-associated toxicity deaths during conventional chemotherapy are needed to further improve the survival of patients with neuroblastoma aged less than 1 year.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.3 no.2
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• pp.151-159
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• 2000

Purpose: Recently, a wide application of gastrofiberscopy in the pediatric group have revealed that nodular duodenitis is not an uncommon disease in children and is suspected to be associated with H. pylori infection. The aim of this retrospective study was to investigate the clinical and histopathologic features in children with nodular duodenitis, and to assess the correlations beween both. Methods: During a period of 5 years (Jan. 1995~Dec. 1999), we investigated clinical, endoscopic and histopathologic features of 39 consecutive patients diagnosed as having nodular duodenitis at Pediatric department of Seoul Red Cross Hospital. In 35 children with nodular duodenitis endoscopic biopsy specimens were stained with Hematoxylin & Eosin and Giemsa's stain, and were graded according to the criteria outlined by Triadafilopoulos, Whitehead et al., and Prieto et al.. Statistical analyses were performed with Graph PAD InStat. Results: The prevalence rate of nodular duodenitis was 17.1% and the most frequent chief complaint was abdominal pain (69.2%). Endoscopically grade 1 was the most common (45.7%) and nodular gastritis was coexistent in 28.3%. The most common histology of the duodenum was grade 2 (54.3%), and the most common histologic score of the stomach was 2 (42.9%). H. pylori was found in the duodenum in 37.1%, and in the stomach in 31.4%. The correlation coefficient between the endoscopic grade and the histologic grade of nodular duodenitis was 0.3983 (p=0.0178). And the correlation coefficient between the histologic grade and the grade of H. pylori colonization in the duodenum was 0.5154 (p=0.0018). Conclusion: There was significant correlation between the endoscopic grade and the histologic grade of nodular duodenitis, and was also significant correlation between the histologic grade and the grade of H. pylori colonization in the duodenum. Therfore H. pylori infection should be regarded as an etiologic factor of nodular duodenitis.

• Pediatric Infection and Vaccine
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• v.7 no.1
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• pp.83-93
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• 2000

Purpose : Urabe AM-9 strain was known to be associated with increased aseptic meningitis. The reason for high incidence of vaccine-associated meningitis was known that nucleotide(nt) substituted form G to A at position 1081 of the hemagglutinin-neuraminidase(HN) gene and therefore, glutamic acid changed to lysine at amino acid 335. We assessed by comparing nt sequence of the HN gene form Urabe AM-9 strain with wild strain and documented the correlation between nt substitution and vaccine-associated meningitis. Methods : Two lots of Urabe AM-9 vaccine distributed in Korea and mumps wild strains isolated from 1998 through 1999 were analysed. Analysis was made by nt sequencing following amplification of HN gene by RT-PCR. Results : Nucleotide substitution at position 343, 1476, 1570 was not found in both Urabe AM-9 vaccines and wild strains. But analysis of vaccine strains and wild strains isolated from patients revealed substitution from G to A at nt 1081 of the HN gene. Therefore, it encodes lysine instead of glutamic acid at amino acid 335. There was no mixture from of G and A at nt 1081. Nt at 1470 of one lot of Urabe AM-9 vaccines changed from C to A after Vero cell passage. Nt at 1727 of vaccines and wild strains was substituted A to G, so it encodes glycine instead of aspartic acid. Conclusion : Nucleotide analysis of HN gene revealed that nt 1081 of Urabe AM-9 vaccines and wild strains had wild type AAA($Lys^{335}$) instead of variant type GAA($Glu^{335}$). The results of this study suggest that there was a probability of vaccine-associated meningitis due to Urabe AM-9 in Korea before. But incidence of actual side effect was not evaluated because there was no reporting system in Korea.

• 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.

• Journal of Life Science
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• v.17 no.8 s.88
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• pp.1090-1099
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• 2007

Myristicin, l-allyl-3,4-methylenedioxy-5-methoxybenzene, was one of the major essential oils of nutmeg. However, its anti-allergic effect in the Th1/Th2 immune response was poorly understood. Recently, it was shown that T-bet and GATA-3 was master Th1 and Th2 regulatory transcription factors. In this study, we have attempted to determine whether myristicin regulates Th1/Th2 cytokine production, T-bet and GATA-3 gene expression in ovalbumin (OVA)-induced asthma model mice. Myristicin reduced levels of IL-4, Th2 cytokine production in OVA-sensitized and challenged mice. In the other side, it increased $IFN-{\gamma}$, Th1 cytokine production in myristicin administrated mice. We also examined to ascertain whether myristicin could influence eosinophil peroxidase (EPO) activity. After being sensitized and challenged with ovalbumin (OVA) showed typical asthmatic reactions. These reactions included an increase in the number of eosinophils in bronchoalveolar lavage fluid, an increase in inflammatory cell infiltration into the lung tissue around blood vessels and airways, and the development of airway hyper-responsiveness (AHR). The administration of myristicin before the last airway OVA challenge resulted in a significant inhibition of all asthmatic reactions. Accordingly, these findings provide new insight into the immunopharmacological role of myristicin in terms of its effects in a murine model of asthma.

• Korean Journal of Hospice Care
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• v.2 no.1
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• pp.58-74
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• 2002

This paper constitutes a descriptive investigation and used a structured questionnaire to investigate nurses' and doctors' recognition of cancer patients. The subjects were extracted from the medical personnel working at the internal medicine, the surgery ward, the obstetrics and gynecology department, the pediatrics department, the cancer ward, and the emergency room of five general hospitals located in Seoul and Gyeonggi Province. The research lasted from August, 2001 to September 2001. Total 137 nurses and 65 doctors were included and made out the questionnaires directly distributed by the investigator. The study tool was also developed by the investigator and consisted of such items as the demographic and social characteristics, the medical personnel's recognition degree of cancer and cancer patients, their recognition of the management of cancer patients, and their participation in a hospice. The results were analyzed using the SPSS Window program in terms of technological statistics, ranks, t-test, and ANOVA. The reliability was represented in Cronbach' α=.75. The nurses' and doctors' recognition degree of cancer and cancer patients had an overall average of 3.86 at the 5 point-scale. The items that received an average of 4.0 or more included 'Medical personnel should explain about the cancer cure plans to the cancer patient and his or her family', 'A patient whose case has been diagnosed as a terminal cancer should be notified of it, 'If I were a cancer patient, I would want to get informed of it,' and 'Cancer shall be conquered whenever it is'. In the meantime, the items that received an average of 3.0 or less was 'My relationship with the cancer patient's family has gotten worse since I announced his or her impending death.' And according to the general characteristics and the difference test, the recognition degree of cancer and cancer patient was high among the subgroups of nurses, females, married persons, who were in their 30s, who had a family member that was a cancer patient, and who received a hospice education. The biggest number of the nurses and doctors saw 'a gradual approach over several days'(68.8%) as a method to tell a cancer patient about his or her cancer diagnosis or impending death. Those who usually tell tragic news were the physician in charge(62.8%), the family members or relatives(32.1%) and the clergymen(3.8%) in the order. The greatest number of them recommended a cancer patient's home as the place where he or she should face death because they thought 'it would stabilize his or her mentality'(91.9%) while a number of them recommended the hospital because they 'should give the psychological satisfaction to the patient'(40%) or 'should try their best until the last moment of the patient's death'(30%). A majority of the medical personnel regarded 'smoking or drinking' and 'diet' as the causes of cancer. The biggest symptom of a cancer patient was 'pain' and the pain management of a cancer patient was mostly impeded by the 'excessive fear of drug addiction, tolerance to drugs and side effects of drugs' by medical personnel, the patient, and his or her family. The most frequently adopted treatment plan of a terminal cancer patient was 'to do whatever the patient or his or her family wants' to resort to a hospice' and 'to continue active treatment efforts' in the order. The biggest reasons why a terminal cancer patient went to see a doctor were 'pain alleviation' 'control of symptoms other than pain(intravenous supply)' and 'incapability of the patient's family' in the order. Terminal cancer patients placed their major concern in 'spiritual(religious) matter' 'emotional matters' their family' 'existence' and 'physical matters' in the order. 113(58.5%) of the whole medical personnel answered they 'would recommend' an alternative treatment to a terminal cancer patient mostly because they assumed it would 'stabilize the patient's mentality.' Meanwhile, 80(41.5%) of them chose 'not to recommend it mostly due to the unverified effects and high cost of it(78.7%). A majority of them, I. e. 190(94.1%) subjects said they 'would recommend' a hospice to a terminal cancer patient mostly because they thought it would help the patient to 'mentally prepare'(66.6%) Only 17.3% of them, however, had received a hospice education, most of which was done through the hospital duty education(41.4%) and volunteer training(34.5%). The follows are results of this study: 1. The nurses and the doctors turned out to be still passive and experience confusion in dealing with a cancer patient despite their great sense of responsibility for him or her. 2.Nurses and Doctors realize the need of a hospice, but an extremely small number of them participate in a hospice education or performance. Thus, a whole recognition of a hospice should be changed, for which purpose a hospice education for nurses and doctors should be provided. 3.Terminal cancer patients preferred their home to a hospital as the place to face their impending death because they felt it would bring 'mental stability.' And most of nurses and doctors think it would be unnecessary for them to be hospitalized just for control of their symptoms. Accordingly a terminal cancer patient can be cared at home, and a home hospice care needs to be activated.

• Childhood Kidney Diseases
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• v.6 no.2
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• pp.142-154
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• 2002

Purpose : One of the most important adverse effects of long-term cyclosporine therapy is nephrotoxicity, the morphologic changes of which include interstitial fibrosis and arteriolar hyalinization. Recently, several authors have shown that osteopontin plays an important role in the development of interstitial fibrosis by acting as a macrophage chemoattractant and stimulating the production of $TGF-{\beta}$ in experimental cyclosporine nephrotoxicity. However, the relationship between osteopontin and $TGF-{\beta}$ in humans has not been clearly documented so far. We studied the expression of osteopontin and $TGF-{\beta}$ in children with minimal change nephrotic syndrome treated with cyclosporine to demonstrate whether there is a relationship between cyclosporine toxicity and osteopontin expression as previously shown in animal models. Materials and methods : Nineteen children (15 males and 4 females) were the subject of this study. Renal biopsies had been performed before and after the cyclosporine therapy (mean duration: 15.9 months). In 5 patients, additional biopsies were performed after completing the cyclosporine treatment (mean; 26 months). The expressions of osteopontin and $TGF-{\beta}$ were evaluated by immunohistochemistry in the glomeruli and tubulointerstitium. Results : Osteopontin expression was significantly increased in the glomerular mesangium and tubules after cyclosporine treatment. But there was no statistically significant increase of $TGF-{\beta}$ in the interstitium. There was no significant increase in tubular osteopontin and interstitial $TGF-{\beta}$ expression in those cases developing interstitial fibrosis after cyclosporine treatment compared with cases those not developing interstitial fibrosis. No significant changes in osteopontin or $TGF-{\beta}$ expression were observed in subsequent 5 biopsy samples after discontinuation of cyclosporine compared with the first follow up biopsies. Conclusion : These results suggest that osteopontin is a nonspecific marker of renal injury rather than a mediator of interstitial fibrosis in cyclosporine nephrotoxicity of human.

• Childhood Kidney Diseases
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• v.6 no.2
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• pp.209-217
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• 2002

Purpose : Corticosteroid has been used as the mainstay therapy of childhood NS. But SIO is one of the serious complications of long-term steroid therapy, especially in growing children. Recently calcium, calcitonin, PTH, vitamin D and bisphosphonate has been used to treat or prevent SIO in adult, which is rare in children with NS. We studied the effect of $1{\alpha}-(OH)D_3$ and Pamidronate on SIO using dual energy X-ray absorptiometry (DEXA). Patients and methods : We studied thirty patients who admitted in the Dept. of Pediatrics of Kyung Hee Medical Hospital with NS. All patients was received longterm steroid therapy. There was no history of bone, liver, or endocrine disease. The samples, serum protein, albumin, BUN, creatinine, calcium, phosphorus, and BMD were obtained before and the six months after the dose of $1{\alpha}-(OH)D_3$ and Pamidronate, respectively Results : The mean age was $6.9{\pm}3.3\;and\;6.5{\pm}2.5$ years old. The mean duration of steroid therapy was $28.8{\pm}1.8\;and\;27.6{\pm}1.0$ months. The changes of serum protein, albumin, BUN, creatinine, calcium and phosphorus level between pre-treatment and post-treatment did not show statistical significance in both $1{\alpha}-(OH)D_3$ and Pamidronate treatment group. However, BMD was increased in both from $0.472{\pm}0.12\;and\;0.457{\pm}0.10\;g/cm^2\;to\;0.533{\pm}0.12$ and $0.529{\pm}0.09\;g/cm^2$ after treatment. (P<0.05) Conclusion : Both $1{\alpha}-(OH)D_3$ and Pamidronate appears to be effective in treating and preventing SIO in children with nephrotic syndrome requiring long-term steroid therapy.

• Childhood Kidney Diseases
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• v.3 no.1
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• pp.88-94
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• 1999

Purpose : Malformation of urinary tract is among the most common of all congenital anomalies and can progress to irreversible renal damage before diagnosis due to difficulty of early diagnosis. Present study was undertaken to determine the clinical characteristics of urinary tract anomaly and to find out the most appropriate diagnostic and therapeutic measures for children with these anomalies. Methods : During the past 10 years from 1987 to 1998, review of medical records revealed 65 children with congenital anomaly of urinary tract and the following results were obtained. Results : The most common anomalies were ureteropelvic junction obstruction occuring in 26 cases ($36\%$), followed by ureteral duplication in 11 cases, renal agenesis in 10 cases and ureterovesical function obstruction in 7 cases. Complex anomaly of urinary tract was found in 8 cases and anomaly of other systems such as congenital heart disease was detected in 11 cases. The most frequent age group at the time of diagnosis was below 1 year of age constituting 39 cases ($60\%$) and male preponderance was noted as male to female ratio being 2.25:1. Presenting symptoms were urinary tract infection in 25 cases, followed by hematuria, abdominal mass, abdominal pain and voiding difficulty, etc, and in 11 cases, the anomaly was picked up by routine prenatal ultrasonography. Azotemia was noted in 9 cases and the underlying anomaly was obstructive uropathy in 4 out of these 9 cases. Surgical correction was undertaken in 38 cases (most frequently in cases of obstructive uropathy) and in 2 out off cases with obstructive uropathy in whom surgical correction was done, azotemia disappeared during follow up period of 1-5years. No new cases of deteriorating renal function appeared during follow-up period. Conclusion : In spite of high incidence of congenital malformation of urinary tract, early diagnosis is still hampered by nonspecific symptoms and signs. Therefore, in patients with symptoms such as urinary tract infection, abdominal pain and voiding problems, etc, it Is advisable to take various diagnostic tests promptly to pick up any urinary tract anomaly and to apply proper therapy in order to avoid progression to irreversible renal damage. In this regard, prenatal ultrasonography should be utilized more widely as a routine procedure to detect any urinary tract anomalies before birth.