Purpose : The incidence of type 2 diabetes in children and adolescents has been reported to increase recently. The aim of this study is to investigate the clinical features of type 2 diabetes developing during childhood and adolescent period. Methods : The medical records of 33 patients with type 2 diabetes were reviewed. We analysed clinical manifestations, demographic data, and modes and responses of treatment. Results : Age at diagnosis was $13.4{\pm}1.8$ years. Seventy percent of patients revealed pubertal signs at diagnosis. Half of the patients had BMI more than $25kg/m^2$. Seventy-three percent of patients had family history of type 2 diabetes. Acanthosis nigricans were found in 18% of patients. Nineteen(57.6%) patients were diagnosed incidentally by random urine or blood glucose test without any typical diabetic symptom or sign. The modes of therapy to control hyperglycemia were insulin alone(75.8%), oral hypoglycemic agents alone(9.1%), insulin and oral hypoglycemia agents(9.1%), and only diet with exercise(6%). At the time of investigation, 45.5% of patients were not using insulin. The typical diabetic symptoms at diagnosis were more prevalent in patients who required insulin for more than two years than patients who did not(P<0.05). Conclusion : The development of type 2 diabetes in children and adolescents is possibly related to puberty, obesity, family history, and defects in insulin secretion rather than insulin resistance. Many children and adolescents with type 2 diabetes required insulin initially and some of them could discontinue. More than half of the patients were diagnosed as diabetes without any typical symptom or sign, which might be one of the predictive factors of the prolonged insulin requirement.
Purpose : The aim of our study was to evaluate the therapeutic response to cyclosporine, time to remission and side effects in steroid resistant nephrotic syndrome (SRNS). Methods : This study included 22 children with idiopathic SRNS who were treated with cyclosporine between June 1989 and August 2006. Medical records were reviewed retrospectively. Results : The mean age of patients at diagnosis was $5.2{\pm}3.3\;years$. The male to female ratio was 1.2:1. Pre-treatment renal biopsies showed minimal change (MCD) in 12 (54.5%), focal segmental glomerulosclerosis (FSGS) in 8 (36.4%), membranous nephropathy (MGN) in one (4.5%) and mesangioproliferative glomerulonephritis in one (4.5%). 15 (68.2%) patients responded to cyclosporine, of whom 11 (91.6%) patients were MCD, 3 (37.5%) patients FSGS, and 1 patient MGN (MCD vs FSGS, P<0.05). The time to remission in patients who responded to cyclosporine was $31.5{\pm}15.2\;days$. Four of the 15 cyclosporine responders maintained complete remission even after cessation of the medication Seven still received cyclosporine, 2 were intermittently treated with steroids after discontinuation of cyclosporine, and two were treated with cyclosporine and steroids. The mean duration of cyclosporine therapy was $546.5{\pm}346.2$, $1,392.9{\pm}439.7$, $439.5{\pm}84.1$, and $433.5{\pm}74.2$ days, respectively. We performed post-treatment biopsies in 8 patients and partial interstitial fibrosis and tubular atrophy were found in two. Conclusion : The thrapeutic response of cyclosporine is good in steroid resistant nephrotic syndrome, especially in minimal change. But, there is a problem of long term cyclosporine dependency.
Nam, Sook Hyun;Son, Young Bae;Lee, Bo Lyun;Lee, Jeehun;Ki, Chang-seok;Lee, Munhyang
Clinical and Experimental Pediatrics
/
v.50
no.9
/
pp.868-874
/
2007
Purpose : We performed this study to investigate the perinatal and developmental features of the patients with congenital myotonic dystrophy (CDM) confirmed by the molecular genetic method and the clinical characteristics of their mother, and to identify the relation between the number of CTG repeats and the clinical severity.Methods : A retrospective review of the medical records and the results of the dystrophia myotonica protein kinase (DMPK) gene test was done for the patients who were confirmed as CDM through gene analysis from January 2001 to September 2006. Results : All of the eight patients (male 2, female 6) showed moderate to severe degree of perinatal distress and feeding difficulty associated with profound hypotonia. Three patients had the history of polyhydramnios and two patients had equinovarus deformity. The developmental milestones were delayed in all patients, which improved gradually with age. All of their mothers demonstrated myotonic symptoms and typical myopathic face. The number of CTG repeats in DMPK gene analysis ranged 1,000-2,083, and there was no significant correlation between the number of CTG repeats and the time of walking alone. Conclusion : All patients with CDM presented with severe hypotonia in perinatal period, and developmental delay thereafter, which were improved with age. All of their mothers manifested myotonic symptoms with typical myopathic face, and the identification of such features greatly contributed to the diagnosis of the patients. The number of CTG repeats had no significant influence on the motor development.
Purpose : Prader-Willi syndrome (PWS) is a complex genetic disorder, caused by the deletion of the paternally derived 15q11-13 region or the maternal uniparental disomy of chromosome 15 (mUPD(15)). In this study, we compared phenotypic differences between those patients whose disease was caused by microdeletion and those caused by mUPD(15). In addition, a comparison of the efficacy of growth hormone (GH) therapy between these two PWS genotypes was analyzed. Methods : Fifty-three patients were diagnosed as having PWS based on molecular and cytogenetic analyses and clinical features. Data that included maternal age, birth weight, a feeding problem in the neonatal period, cryptorchidism, developmental delay or mental retardation, short stature, hypopigmentation, changes in height, weight, and body mass indexes (BMI) before and after GH treatment were obtained by a retrospective review of medical records. The data from the patients with microdeletion were compared with those from the patients with mUPD(15). Results : Of the 53 patients with genetically confirmed PWS, 39 cases had microdeletion and 14 mUPD(15). Maternal ages were significantly higher in the mUPD(15) group, and hypopigmentation and a feeding problem in the neonatal period were more frequent in the microdeletion group. Growth hormone was administered to 20 patients [14 with microdeletion, 6 with mUPD(15)]. There were no differences between the two groups in height velocity, weight and height SDS, and BMI after GH therapy. Conclusion : Phenotype and genotype correlations were observed in Korean PWS patients, such as more advanced maternal ages in the mUPD(15) group and more feeding problems and hypopigmentations in the microdeletion group. Further long-term prospective studies are needed to correlate other aspects of the phenotypes.
Seo, Hye-Eun;Lee, Ji Hye;Kim, Ji Yoon;Lee, Dong Ha;Lee, Heung Kyo;Lee, Kun Soo
Clinical and Experimental Pediatrics
/
v.50
no.9
/
pp.875-881
/
2007
Purpose : Chromosome analysis is important in genetic study and genetic counseling. This study was performed to evaluate the type and incidence of chromosome abnormalities in a single hospital for 25 years. Methods : Chromosome analyses were performed on peripheral blood lymphocytes, obtained from 4,856 patients with suspected chromosomal aberrations, referred to cytogenetic laboratory in Department of Pediatrics, Kyungpook National University Hospital from May 1981 to October 2005. Results : We analyzed 4,567 cases. Children were 3,014 cases (66.0%) and adult were 1,553 cases (34.0 %). The most common purpose of the chromosomal analysis was growth and developmental abnormality in children and infertility in adults. Total chromosomal aberration rate was 16.9% (770/4,567). Among those cases, the numerical abnormalities were 12.2% (558 cases), the structural abnormalities were 4.1% (187 cases), and others were 0.5% (25 cases). The relative frequencies of autosomal abnormalities were 6.4% (294 cases) in Down syndrome; 0.2% (7 cases) in Edwards syndrome; 0.1% (4 cases) in Patau syndrome; 0.2% (10 cases) in other abnormalities, of sex chromosome, 2.9% (131 cases) in Klinefelter syndrome; 2.2% (99 cases) in Turner syndrome; 0.2% (8 cases) in 47, XXX; 0.1% (3 cases) in 47, XYY. Among the structural abnormalities, translocation was 1.8% (84 cases), inversion was 0.8% (37 cases), deletion was 0.4% (17 cases), and insertion was 0.3% (13 cases), in order of frequency. Conclusion : In this study, the type, incidence and distribution of cytogenetic abnormalities by karyotype were reviewed. We hope that our study could be used as a basic information on the diagnosis, treatment and genetic counseling for chromosome abnormalities in Korea.
Purpose : Allergic proctocolitis is a major cause of bloody stool in early infancy. This study was aimed at ascertaining the clinical courses, sigmoidoscopic and histologic findings of allergic proctocolitis. We also analyzed the relationship between peripheral eosinophilia, the age at symptom onset, and sigmoidoscopic and histologic findings. Methods : We reviewed 25 infants retrospectively who had sigmoidoscopy and biopsy performed with a clinical diagnosis of allergic proctocolitis from April 2003 to April 2007. Results : The mean age at symptom onset was $15.2{\pm}13.2$ weeks. Fourteen infants (56.0%) were breast fed, one (4.0%) was formula fed, six (24.0%) were on combined formula, and four (16.0%) were on a weaning diet. Peripheral eosinophilia (${\geq}250/mm^3$) was seen in eighteen infants (75.0%), but total serum IgE was increased only in six (24.0%). Sigmoidoscopic findings were variable from normal (8.0%), erythema or edema (20.0%), lymphoid hyperplasia (8.0%), erosion (12.0%), hemorrhage and ulcer (4.0%) to lymphoid hyperplasia with erosion, hemorrhage, or ulcer (48.0%). Histologic findings showed focal infiltration of eosinophils in lamina propria (96.0%) and crypt epithelium (96.0%). In twenty four infants (96.0%), the number of eosinophils in mucosa was increased by a more than 60/10 high power field. There was a negative correlation between peripheral eosinophilia and the age at symptom onset. Among the twelve breast fed infants, bloody stool disappeared in ten (83.0%) with a maternal elimination diet of major food groups, but two improved spontaneously. Conclusion : Allergic proctocolitis should be considered as one of the major causes of bloody stool in healthy appearing infants. To confirm the diagnosis it is necessary to perform sigmoidoscopy and biopsy but histologic findings are more informative than sigmoidoscopic findings. Peripheral eosinophilia was prominent in the infants with an early onset of symptoms. Most infants experienced benign courses and recovered with the elimination of causative foods but did not need exclusive food restrictions.
Journal of the Korea Organic Resources Recycling Association
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v.9
no.4
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pp.125-134
/
2001
The purpose of this study is to provide basic information for a future countermeasure municipal and to establish several wastes policy after investigating solid wastes from Sunchon City. In addition, this research can be supported to manage of recycling plant and to reuse plant of each wastes. Results are as bellows after checking up and analysis type of waste in Sunchon city Unit solid waste generation rate from single family is $0.50kg/person{\cdot}day$, and total solid wastes are 41.9ton/day. Unit solid waste generation rate from apartments is $0.45kg/person{\cdot}day$, and solid wastes generation is 55.5ton/day. Unit solid waste generation rate from agricultural is $0.22kg/person{\cdot}day$ and total solid wastes are 13.5ton/day. That show total amount of municipal solid wastes from residential are 110.9ton/day. Unit solid waste generation rate from traditional markets is $1.85kg/person{\cdot}day$, and solid waste total volume is 5,400kg/day. Unit solid waste generation rate from small store is $2.03kg/person{\cdot}day$, and solid waste total are 25,101kg/day. Therefore, this show that total wastes are 30.50kg from downtown and commercial area. Solid waste quantity from Industrial area (Factory region) is 8.5ton and in case of school and hospitals are 7.2kg/day and 3.0kg/day. Solid waste amount from Institutional is 6.6kg/day. Food wastes were eliminated from municipal solid wastes as standard 63.4ton/day, and combustible wastes were 126.9ton/day. If it schedule about 5 years (by 2006) as durable year for food wastes treatment plant, it is expected 42.5ton/day for treatment capacity. We can judge that it is effective to be set 2 lines equipment ${\times}25ton/day$ as treatment ability under considering unexpected working condition such as any repair, trouble and an electrical load. If it schedule about 10 years (by 2011) as durable year for food wastes treatment plant, it is expected 150 ton/day for treatment capacity. We can conclude that it is effective to be set 2 lines equipment ${\times}80ton/day$ as treatment ability under considering working condition such as low loaded operating and the repair for incineration.
Bone metastases represent an important and frequent clinical problem in patients with advanced cancers. Especially, painful bone metastases are common features in these patients. Radiotherapy is an effective tool for palliative aim of painful metastatic osseous lesions. Various treatment results have been previously reported. The present retrospective study was aimed to evaluate the efficacy of palliative irradiation on pain relief, with the goal of selecting appropriate irradiation dose schedule. Radiotherapy consisted of 5times a week with a various fractional dose between 180 and 400cGy. The response of pain relief and the survival time after completion of radiotherapy are related to total dose and most of the patients have shown a similar response by the end of radiotherapy. The higher dose and the more aggressive multimodality treatment, the better pain control and the longer survival time.
Infantile hypertrophic pyloric stenosis(IHPS), which occurs three of 1,000 live births, is a major cause of nonbilious vomiting of early infancy but its etiology and pathogenesis are still obscure. The operation of pyloromyotomy as described by Ramstedt in 1912 remains the standard of care for the treatment of IHPS. From January 1993 to October 1996, 35 infants with IHPS were surgically treated and the following results were obtained. 1. Thirty-five patients comprised 32 males and 3 females, and the ratio of male to female was 10.7:1. 2. The most prevalent age group was between 2 weeks and 8 weeks. 3. Of 35 infants, first born babies were 23 cases(65.7%). 4. Breast feeding was in 23 cases(65.7%). 5. The body weight percentile at admission was lower than 50 percentile in all 35 cases. 6. Onset of symptoms was predominantly between 1week and 2 weeks in 11 cases(31.4%). 7. All had a history of nonbilious vomting, generally projectile in nature. 8. In the measured serum electrolytes, hypokalemia was noted in 9 cases(25.7%), hypochloremia was observed in 14 cases(40.0%). 9. In the preoperative ultrasonography, the average muscle thickness, diameter, and length of the pylorus were 6.2mm, 12.1mm, and 17.9mm, respectively. 10. Among 35 cases, significant sonographic criteria of IHPS, muscle thickness of 5mm or more, were noted in 26 cases(83.9%). 11. A total of seven associated anomalies were noted in six patients. 12. All 35 cases were treated with Fredet-Ramstedt pyloromyotomy. 13. There were postoperative complications of wound infection in 2 cases. Intermittent nonprojectile vomiting was presented in 8 cases(22.9%) after operation, but one of them was relieved in 13 days and the rest were relieved within one week by adjustment of oral intake.
We compared the efficacy of each modality of treatment group in reducing the frequency of coronary artery abnormalities and changes of clinical courses in children with kawasaki disease in the children of 81 cases who were admitted in pediatric department of Yeungnam University Hospital from September 1985 to August 1990, Group A(37 cases)-aspirin alone, Group B(44 cases)-intravenous gammaglobulin(400mmg/kg/day) for 5 consecutive days, plus aspirin. We studied the frequency of echocardiographic abnormalities, the duration of fever, and changes in the total white blood cell counts, platelet counts, ESR and CRP value at 1, 2 and 3 weeks of the illness and compared the results between the two groups. The results were as follows, 1) There as no significant intergroup difference in age and sex ratio. 2) The duration of the febrile period after the initiation of the therapy was significant shorter in group B($2.5{\pm}1.2$ days) than in group A:($5.2{\pm}3.5$ days)(p<0.01). 3) No significant difference was noticed in the WBC and platelet counts in two groups as measured at admission day, 1 and 2 weeks of the illness. However, at 3weeks of illness significant difference was noted. 4) The CRP values measured at 1, 2 and 3 weeks after treatment were significantly lower in group B($2.42{\pm}1.8$, $2.00{\pm}1.2$, $1.16{\pm}1.0$) than in group A($7.22{\pm}5.3$, $5.25{\pm}3.9$, $1.85{\pm}1.2$) respectively(p<0.01). 5) In 2D- Echocardiogram, coronary artery dilataton was more frequent in Group A than in Group B at 6month of illness(p<0.01). In conclusion, intravenous gammaglobulin therapy was effective in the shortening of the duration of fever and in the antiinflammatory action and somewhat effective in prevention of coronary artery aneurysm.
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