• Childhood Kidney Diseases
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• v.13 no.2
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• pp.146-152
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• 2009

Purpose : We performed this study to evaluate the incidence and clinical significance of antiphospholipid antibodies (aPL Ab) in Korean children with Henoch-$Sch{\ddot{o}}nlein$ purpura (HSP). Methods : The medical records of 62 patients (31 boys and 31 girls) aged $46.0{\pm}3.1$ (1-16) years with a clinical diagnosis of HSP based on the EULAR/PReS criteria were reviewed retrospectively. From the years 2007 to 2009, the sera from children with acute HSP were tested for aPL Ab such as LA, anti-cardiolipin antibody and anti-${\beta}_2$ glycoprotein I antibody. Results : LA was positive in 18 (29%) of the 62 patients with HSP and We divided the patients into the two groups LA positive group (N=18) and LA negative group (N=44). There were no significant differences between the two groups with regard to abdominal pain, arthralgia and renal involvement, but LA positive group had significantly higher C-reactive protein ($4.3{\pm}7.2$ mg/dL vs. $1.3{\pm}1.8$ mg/dL, P=0.035), erythrocyte sedimentation rate ($37.5{\pm}26.2$ mm/hr vs. $25.1{\pm}22.6$ mm/hr, P= 0.039), IgM ($148.1{\pm}48.4$ mg/dL vs. $114.9{\pm}41.5$ mg/dL, P=0.024), C3 ($143.1{\pm}21.9$ mg/dL vs. $129.7{\pm}24.5$ mg/dL, P=0.048) and C4 levels ($30.9{\pm}6.3$ mg/dL vs. $24.9{\pm}7.8$ mg/dL, P=0.002) compared with LA negative group. Conclusion : We found that the incidence of positive aPL Ab tests was relatively higher in Korean children with HSP and the presence of aPL Ab was associated with acute inflammatory process of HSP. These results suggest that the aPL Ab are involved in the pathogenesis of HSP in children.

• Clinics in Shoulder and Elbow
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• v.13 no.1
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• pp.58-63
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• 2010

Purpose: To evaluate clinical results of a single percutaneous injection of platelet-rich plasma in patients with refractory lateral epicondylitis. Materials and Methods: Between Jan and Dec 2009, fifteen patients (5 male, 10 female) received a diagnosis of lateral epicondylitis of the elbow and were evaluated in this study. Their average age was 43.5 years. All patients were initially given a variety of non-surgical treatments for more than 1year. All patients were considering surgery. These patients were given a single percutaneous injection of 3cc of platelet-rich plasma. To assess pain, we used a visual analogue scale (VAS) at rest and during work & the Patient-Rated Tennis Elbow Evaluation (PRTEE) score. We compared the score before treatment with scores 4 and 12 weeks after treatment. Results: Average VAS scores at rest improved from 4.6 before treatment to 2.5 at week 4, and 1.8 at week 12. The average VAS score while working also improved from 7.8 before treatment to 6.2 at week 4, and 4.25 at week 12. The average PRTEE score improved from 60.13 before treatment to 46.12 at week 4 and 24.6 at week 12. Conclusion: Treatment using a single percutaneous injection of platelet-rich plasma in patients with refractory lateral epicondylitis appears to be an effective treatment modality. Platelet-rich plasma should be considered before surgical intervention.

• Clinical and Experimental Pediatrics
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• v.50 no.9
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• pp.868-874
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• 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.

• Clinical and Experimental Pediatrics
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• v.50 no.9
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• pp.891-895
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• 2007

Purpose : Acute disseminated encephalomyelitis (ADEM) is a demyelinating disease of the central nervous system and mostly develops after viral illness or vaccinations. We investigated the clinical differences and neurologic outcomes according to the distribution of the lesions on brain MRI. Methods : The study group was composed of 21 patients from January 1995 to August 2003 in Kyunghee University hospital. We grouped the patients according to the MRI findings as follows. Group I (14 cases): Multi- or unifocal lesions only in the cerebral white matter. Group II (7 cases): lesions in the gray matter with or without white matter involvement. Results : 1. Preceding events were as follows: no defined prodrome (38.1%), upper respiratory tract infection (28.6%), nonspecific febrile illness (19.0%), gastointestinal disturbance and vaccination. 2. Presenting symptoms were as follows: seizures (76.2%), headache/vomiting (47.6%), altered consciousness (38.1%), hemiparesis, cerebellar ataxia, visual disturbance and facial nerve palsy. 3. Laboratory findings were as follows: CSF pleocytosis (76.2%), leucocytosis (38.1%) and elevated CSF protein (28.6%). 4. Fifteen patients were recovered completely without neurological sequelae. Three patients in group I and 1 patient in group II had intractable seizures. Two patients in group I and 2 patients in group II had motor disturbance. Conclusion : There were no statistically significant differences in preceding events, presenting symptoms, and neurological outcomes according to the distribution of the lesions on brain MRI. However, the ADEM have quite diverse clinical manifestations and neuroimage findings. MRI plays an important role in making diagnosis of the patients who are suspected of ADEM.

• Clinical and Experimental Pediatrics
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• v.50 no.9
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• pp.875-881
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• 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.

• Journal of Yeungnam Medical Science
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• v.10 no.1
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• pp.68-76
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• 1993

Pregnancy induced hypertension is multifaceted syndrome with variable involvement of several key organ systems, so sensitive and specific laboratory tests for predicting severity and prognosis, and early diagnosis of this disease are required. Because heme catabolism results in equimolar production of carboxyhemoglobin, iron and bilirubin, a concomittant rise of these parameters would provide confirmation of increased heme catabolism. Microangiopathic hemolytic anemia may occurs in severe preeclampsia, but it is not known whether increased red cell turnover occurs with mild preeclampsia as complication. The purpose of this study was to confirm that increased heme catabolism also occurs in patients with mild preeclampsia. The analysis of data was done on 23 cases with mild preeclampsia and 35 normal pregnant women, who were admitted to Yeungnam University Hospital from October 1992 to March 1993. The results were as follows. 1. The mean antepartum serum iron concentration was significantly higher in the group with mild preeclampsia($86.5{\pm}6.1{\mu}g/dl$) than in the controls($53.2{\pm}5.3{\mu}g/dl$). 2. The mean antepartum and postpartum carboxyhemoglobin concentrations were significantly higher in the group with mild preeclampsia(antepartum : $2.55{\pm}0.42{\mu}g/dl$, postpartum : $1.21{\pm}0.4{\mu}g/dl$) than the controls(antepartum : $0.61{\pm}0.2{\mu}g/dl$, postpartum $0.53{\pm}0.2{\mu}g/dl$) 3. During postpartum, carboxyhemoglobin concentration in preeclampsia reduced significantly from antepartum level, but, there was no difference between antepartum and postpartum carboxyhemoglobin concentrations among controls. 4. Bilirubin concentrations were similar in both groups.

• Clinical and Experimental Pediatrics
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• v.49 no.4
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• pp.410-416
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• 2006

Purpose : Ciliary abnormalities of the respiratory system usually accompany recurrent or persistent respiratory diseases such as paranasal sinusitis, bronchiectasis, rhinitis, and/or otitis media, since they cause certain derangements in ciliary cleaning activities. This disease is usually inherited by autosomal recessive trait, but may also be found to be acquired or transient in rare cases after heavy exposure to pollutants, cigarette smoking or severe infection. We performed this study in children with frequently recurrent or persistent respiratory diseases to clarify if the ciliary abnormalities are preceding factors. Methods : We enrolled 17 children with suspected respiratory ciliary abnormalities. The indications for evaluation of ciliary ultrastructure were recurrent or persistent respiratory infections. Children with immunologic abnormalities were excluded. From August 2000 to July 2003, we performed a biopsy on nasal mucosa and examined the structure of ciliary status by using an electron microscope. Results : Of the subjects, there were seven males and 10 females, aged 2 to 10 years. Out of the 17 subjects, 12 cases of chronic paranasal sinusitis, nine chronic coughs, nine frequent upper respiratory infections, seven cases of recurrent otitis media, four cases of recurrent pneumonia, and four cases of bronchial asthma were found. Out of the 17 cases on which histologic examinations were conducted, four cases showed pathologic findings, including one case of inner dynein arm defect, one of microtubular transposition, one of supernumerous tubules, and one singlet, respectively. Conclusion : It is essential for differential diagnosis and effective treatment to identify the abnormalities of ultrastructure of nasal cilia in children with symptoms of frequently recurrent or persistent respiratory diseases, if immunodeficiency or respiratory allergy could be excluded.

• Tuberculosis and Respiratory Diseases
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• v.60 no.2
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• pp.221-227
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• 2006

Background : Cough may be a consequence of bronchial hyperresponsiveness or inflammation. Empirical treatment is important in this context because it difficult to verify the obvious cause of cough using laboratory tests, Corticosteroid has a nonspecific anti-inflammatory effect, and can be used for cough management. However, its response rate has not yet been fully elucidated. This study investigated the short- term effects of inhaled corticosteroid on chronic cough Methods : Patients with chronic cough with a normal chest radiograph and a pulmonary function test were enrolled. Cases with a prior respiratory infection within 8 weeks, a history of bronchial asthma, objective wheezing on examination, subjective symptoms of gastroesophageal reflux or taking an ACE inhibitor were excluded. On the first visit, a methacholine bronchial provocation test, spontaneous sputum eosinophil count performed twice and a paranasal sinus radiograph were checked, and the patients were treated with budesonide turbuhaler $800{\mu}g/day$ for ten days. The primary outcome measure was a decrease in the cough score after treatment. Results : Sixty nine chronic coughers were finally analyzed. The final diagnoses by the routine tests were as follows: bronchial asthma 13.0%, eosinophilic bronchitis 18.8%, paranasal sinusitis 23.2% and non-diagnostic cases 53.6%. The following responses to the inhaled corticosteroid were observed: definite responders, 76.8%, possible responders, 2.9% and non-responders, 20.3%. The response rate was not affected by the final diagnosis even in the non-diagnostic cases. There were minimal adverse drug related effects during the empirical treatment. Conclusion : Routine objective tests such as methacholine provocation, sputum eosinophil count and simple radiographs were notare not suitable for diagnosing chronic cough Therefore, empirical treatment is important. Short term inhaled corticosteroid is effective and can guide a further treatment plan for chronic cough.

• Tuberculosis and Respiratory Diseases
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• v.47 no.5
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• pp.609-617
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• 1999

Background: The aims of this study were to assess the etiologies, survival and prognostic factors of patients with chronic cor pulmonale visited Pusan National University Hospital. Methods : This study included 103 patients with chronic cor pulmonale. There were 67 men and 36 women. The diagnosis of chronic cor pulmonale was primarily based on the presence of underlying lung disorder and echocardiographic finding of enlarged or hypertrophied right ventricle. Other clinical data including patients' symptoms and signs, findings of arterial blood gas analysis, hematologic and biochemical laboratory and pulmonary function test were assessed. Results: The most common underlying lung disorder was pulmonary tuberculosis(59.2%) and chronic obstructive pulmonary disease was the next(28.2%). The survival rate was 57% in one year, 45% in two years, and 34% in three years. The prognostic factors were maximal voluntary ventilation(MVV), forced vital capaoity(FVC), $FEV_1$ serum Na, vital capacity(VC), serum albumin and peak expiratory flow(PEF) in univariate analysis. And in multivariate analysis, serum albumin(p=0.0144) and VC(p=0.0078) were statistically significant. Conclusion: Pulmonary tuberculosis was the most important underlying lung disorder in chronic cor pulmonale. The survival rate was 57% in one year, 45% in two years, and 34% in three years. Serum albumin (p=0.0144) and VC(p=0.0078) were statistically significant prognostic factors.

• Tuberculosis and Respiratory Diseases
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• v.54 no.3
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• pp.283-294
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• 2003

Introduction : Rapidly growing nontuberculous mycobacteria (RGM) can produce numerous types of manifestations including a pulmonary infection. Managing a pulmonary infection due to RGM is unusually difficult to treat because the organism is invariably resistant to traditional antituberculous drugs and has a varying susceptibility to other antibiotics. The experiences of treatments for a RGM pulmonary infection with various antibiotics are also limited. This study evaluated the clinical manifestations, treatment, and the therapeutic outcomes of a RGM pulmonary infection. Subjects and method : Fifty-four cases with RGM from respiratory specimens were identified between November of 1996 and September of 2002 in the Asan medical center. The medical records and radiographic findings in 20 patients who fulfilled the diagnostic criteria of nontuberculous mycobacteria (NTM) pulmonary disease by ATS guidelines. The clinical, laboratory, and radiological parameters between subgroups. Results : Of the 20 patients, 15 were female. The mean age was 57.7 yrs (${\pm}7.5$), and all of the patients had a history of pulmonary tuberculosis. Most (90%) had an underlying lung disease. The majority of the isolates (80%) were M. abscessus. Chest radiography showed bilateral involvement in 80% of the patients. Bronchiectasis and multiple nodules were the main findings. Cavitation was present in 35% of the patients. Even though 70 % of the patients received antituberculous drugs prior to the correct diagnosis, all of the patients eventually received antibiotics. A mean of 3.5 antibiotics were given for an average of 439 days(${\pm}168$). After completing treatment, nine patients showed improvement after a mean 591(${\pm}311$) days of treatment, whereas the antibiotic treatment was unsuccessful in 2 patients. Conclusion : Many patients with a RGM pulmonary infection show an atypical pattern of radiological findings (bronchiectasis and multiple centrilobular nodules). It is very important to differentiate between M. tuberculosis and NTM and to identify the causative organisms among the NTM because a misdiagnosis can lead to an inappropriate and prolonged treatment. Combined antibiotic treatment yielded promising results, and is recommended for treating patients with a RGM pulmonary infection.