• Tuberculosis and Respiratory Diseases
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• v.46 no.3
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• pp.372-385
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• 1999

Background: The purpose of this study was to examine the causes and pathologic process of chronic non-productive cough as an isolated symptom with a normal spirometry and chest radiograph by investigating clinicopathologic findings. Method: We studied 25 adults with chronic non-productive cough over a 3-week period with a normal chest radiograph and pulmonary function tests without any other symptoms. Clinical assessment, cough score, chest and sinus radiograph, pulmonary function tests, methacholine challenge, allergic skin prick test, and bronchoscopy for bronchial biopsies were performed. Subjects were then treated with prednesolone 20 to 30 mg/day for 1 to 2 weeks. Results: The experimental group was divided into two subgroups-those infiltrated with eosinophils, and those infiltrated with lymphocytes depending on eosinophil and lymphocyte counts, both of which were respectively higher than those of the control group. Eosinophils infiltrated group had mean numbers of eosinophil of 89.8 $cells/mm^3$ while control group's mean was 0.4 $cells/mm^2$(p=0.005). Lymphocyte infiltrated group was 4 patients whose mean was 84.3 $cells/mm^2$ with 28.4 $cells/mm^2$ of control group(P=0.026). In addition, the mean thickness of the basement membrane of experimental group was $14.20{\pm}5.20{\mu}m$ in contrast of control group whose mean was $3.50{\pm}1.37{\mu}m$(P=0.001). With the methacholine challenge test, 7 of the 21 eosinophil infiltrated subjects were diagnosed with cough variant asthma ; the other 14 with eosinophilic bronchitis. Three subjects with eosinophilic bronchitis were atopic positive (21.4%) with the skin prick test In the lymphocyte dominant group, all four subjects were diagnosed with lymphocytic bronchitis. Cough score was improved after steroid treatment in 22 of 25 subjects in the experimental group (88.0%). Conclusion: These results suggest chronic non-productive cough as an isolated symptom with a normal spirometry and chest radiograph was associated with airway inflammation by eosinophil and lymphocyte infiltration. The causes for chronic non-productive cough were eosinophilic bronchitis, cough variant asthma, and lymphocytic bronchitis(written in frequency). They further suggest that therapeutic treatment with steroids can provide effective symptomatic relief.

• Journal of the Korean Academy of Child and Adolescent Psychiatry
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• v.13 no.1
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• pp.139-152
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• 2002

Objectives：This study was conducted to compare the clinical and neuropsychological characteristics by DSM-IV subtypes of attention deficit hyperactivity disorder(ADHD) patients who did not have comorbid psychiatric disorders. Methods：5-15 year old children with ADHD were recruited at psychiatric outpatient clinic of Yeungnam University hospital and the patients with comorbidity or neurological abnormalities were excluded. Finally, total 404 children with ADHD were selected for this study. There were 234 subjects of ADHD-C(57.9%), 156 subjects of ADHD-I(38.6%) and 14 subjects of ADHD-HI(3.5%), who fulfilled the DSM-IV diagnostic criteria. The mean age of the total subjects was 9.63±2.49 years old. The psychopathology, IQ, behavioral problems, neuropsychological executive function were evaluated before pharmacological treatment. The measures were Korean Personality Inventory of Child(K-PIC) for psychopathology, 4 behavioral check lists(ADDES-HV, ACTeRS, CAP, SNAP) for behavioral symptoms of ADHD, K-ABC and KEDI-WISC for IQ and Conner's CPT, WCST, SST for neuropsychological executive functions. Results：1) The prevalence of subtypes was ADHD-C, ADHD-I, ADHD-HI in decreasing order. There was no sex difference of prevalence among three subtypes. The mean age of ADHD-I was older than other subtypes. 2) There was significant differences of psychopathology among subtypes, the ADHD-C and ADHD-HI had higher than the ADHD-I in the scores of delinquent, hyperactivity and psychosis；the ADHD-C had higher than the ADHD-I in the scores of family relation and autism, the scores of ego resilience were lower than the ADHD-I. However, there was no difference in anxiety, depression and somatization scores among them. 3) The results of behavioral symptom check lists, the ADHD-C had higher the score of inattention, hyperactivity and impulsivity than the ADHD-I. Meanwhile the results of ACTeRs, which rated by the teachers, were different. 4) There were significant differences of sequential processing scale and arithmetics among subtypes in IQ using K-ABC, but there was no significant difference between the ADHD-C and the ADHD-I after excluding the ADHD-HI due to small numbers. 5) There was numerical difference among subtypes but did not reach statistical significance in three neuropsychological executive function tests. Conclusion：In conclusion, our results revealed that there was significant difference in clinical features among three subtypes but, no significant difference in executive functions.

• Clinical and Experimental Pediatrics
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• v.52 no.2
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• pp.194-198
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• 2009

Purpose : Active perioperative intervention and improvement on surgical technique has decreased the mortality rate of total anomalous pulmonary venous connection (TAPVC); however, when complicated with pulmonary venous obstruction, operative mortality is still high. The purpose of this study was to investigate the clinical course of TAPVC. Methods : Twenty-seven patients who were diagnosed with TAPVC (without other complex heart anomalies) by echocardiogram at Kyungpook National University Hospital from January 1994 to February 2008 were included. Results : Mean age at diagnosis was $28.1{\pm}33.4$ days (1-126 days). Sites of drainage were supracardiac type (15), cardiac (6), infracardiac (5), and mixed (1). Seven patients had pulmonary venous obstruction: 5 with supracardiac type, 1 with cardiac, and 1 with infracardiac. Intraoperative trans-esophageal echocardiograms were performed in 14 patients (58.3%). The operative mortality was 16.7% (4 of 24) and overall hospital mortality (including deaths without operation) was 22.2% (6 of 27). There were 5 postoperative pulmonary venous obstructions. The sites of obstruction were anastomotic in 3 of 5 (60%) patients, and ostial pulmonary vein in the other 2 (40%) patients. Three patients who presented with anastomotic pulmonary venous obstruction underwent reoperation, but all the patients were found to have pulmonary venous anastomotic obstruction. The other 2 patients with ostial pulmonary vein obstruction who had no significant symptoms were diagnosed by routine echocardiographic examination during follow-up. Conclusion : In TAPVC patients, early diagnosis and aggressive surgical management will improve prognosis, and we must pay attention to early and late pulmonary vein restenosis through intraoperative trans-esophageal echocardiogram and peri- and post-operative echocardiographic follow-up examinations.

• Journal of Korean Orthopaedic Sports Medicine
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• v.6 no.2
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• pp.110-114
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• 2007

Purpose: This study evaluate clinical findings & management of rhabdomyolysis after strenuous activities in military police recruit. Materials and Methods: This study was carried out from June $1^{st}$, 2004 and May $23^{nd}$, 2005. The study subjects were 13 military police recruit patients who were admitted to our hospital with intractable muscle pain and swelling, and had suspicions of Rhabdomyolysis. The patients were given various blood tests (CPK, CK-MB, AST, BUN/Cr, and Electrolyte) and clinically observed. The patients were all males, and their average age was 20 $(19\sim21)$ years. Seven cases were due to push-up exercises, 5 was due to a soccer game, and 1 was due to riot control activities. The patients complained of swelling and tenderness in various parts of the extremities. Four complained of swelling and tenderness in forearm, 3 in upper arm, 1 in shoulder, and 5 in lower extremity. The diagnosis of rhabdomyolysis was made if the patient complained clinical symptom and had a blood CPK level of above 1,000 IU/L at the time of admission. Patients who took medication or had medical problem were excluded from this study. Bone scans were taken of all patients 4 hours after giving 99mTc-MDP 20mCi intravenously. Treatment was bed rest and fluid therapy. Patients who complained of excessive pain were given splint immobilization. Results: The average hospitalization day for the 13 patients was 20 days ($14\sim42$ days). Excluding one patient who exhibited ARF at time of admission, all patients showed a decrease of blood CPK below 1000 IU/L at an average hospitalization time of 8 days ($2\sim11$ days). The patient with ARF recovered after hemodialysis and fluid therapy. Conclusion: Patients complaining of swelling and severe muscle pain after excessive exercise or training should be suspicious of exercise induced rhabdomyolysis, and should be given blood tests and fluid therapy immediately.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.16 no.2
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• pp.79-85
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• 2016

Purpose: This study aimed to analyze genetic mutations, clinical manifestations, and treatment of patients with benign HPA in Korea. Methods: This case series study involved ten HPA patients who were referred to our hospital because of high phenylalanine concentration. We investigated their demographic features, clinical manifestations, and mutations of the PAH gene through direct DNA sequencing. Results: Among ten patients with benign HPA, two pairs of patients were related (father-daughter, mother-daughter relationship) cases, and all of them showed no specific clinical manifestations or notable past history. Their plasma phenylalanine levels ranged between 1.2 and 4.2 mg/dL. In the tetrahydrobiopterin (BH4) loading test, all patients were nonresponsive to BH4. In the confirmation test of PAH mutation analysis, we identified eleven different alleles out of twelve. The most common allele was R53H (c.158G> A). In addition, two novel PAH gene mutations, V423A (c.1268T>C) and V51A (c.152T>C), were identified. Although the patients did not receive any pharmacologic treatment or continuous phenylalanine restriction dietary therapy, their neurocognitive development was normal. Moreover, on serial outpatient follow-up tests, all patients maintained phenylalanine levels below 6 mg/dL. Conclusion: This study is the first in Korea to analyze benign HPA patients. All patients with benign HPA could maintain phenylalanine levels below 6 mg/dL with normal neurocognitive development, without continuous therapy. Therefore, performing mutation analysis and distinguishing benign HPA from phenylketonuria (PKU) are important to help improve life quality in patients with benign HPA by avoiding unnecessary lifelong therapy.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.18 no.2
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• pp.62-67
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• 2018

Hunter syndrome, also known as mucopolysaccharidosis Type II (MPS II), is one of the lysosomal storage diseases caused by a lack of the enzyme iduronate 2-sulfatase (I2S). Lack of the I2S enzyme activity leads to accumulation of the glycosaminoglycans (GAG), causing dysfunction of multiple organs and systems. MPS II is an X-linked recessive disease due to mutation of IDS gene located on long arm of the X chromosome (Xq28). To date, more than 350 mutations of IDS gene have been identified in Hunter syndrome. Phenotypes of MPS II are classified as either severe or attenuated depending on the degree of cognitive impairment. Because the phenotype of MPS II is related to the type of mutation, identifying mutations is useful in predicting prognosis. We recently had a case of MPS II diagnosed by exome sequencing in a 7 month old boy with infantile spasm uncontrolled by AED. He was diagnosed with hearing loss at 2 months of age, and he took vigabatrin and prednisolone to control infantile spasms diagnosed at 3 months of age. At 6 months of age, whole exome sequencing was performed to evaluate the infantile spasm and hearing loss in this patient, and the mutation c.851C>T (p.Pro284Leu) inherited from hemizygous mother was revealed. The results of urine Cetylpyridinium Chloride (CPC) precipitation test, which were negative until 8 months of age, were positive from 9 months of age. We report a case of MPS II diagnosed by exome sequencing and treated through enzyme replacement therapy from 9 months after birth.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.15 no.1
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• pp.9-17
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• 2015

Glycogen storage disease (GSD) type Ia is rare inborn metabolic disorder, caused by glucose-6-phosphatase deficiency. It characterized by hepatomegaly, hypoglycemia, lactic acidosis, hypertriglyceridemia, and hyperuricemia and it is usually manifested in the infantile period. In addition, it is also associated with growth failure, pubertal delay, anemia, platelet dysfunction, osteopenia, and pulmonary hypertension. Hepatocellular adenoma and renal dysfunction are frequent late complications. Delayed diagnosis and inappropriate therapy lead to many complications such as growth failure, osteoporosis, refractory gout, renal failure, hepatocellular carcinoma (HCC), and pulmonary hypertension. Here, two Korean sisters diagnosed with GSD Ia, aged 33 and 36 respectively, were described and compared to recent articles about four adults with late diagnosis of GSD Ia. One sister had typical manifestations of GSD Ia including short stature (height, 145 cm), multiple hepatic adenoma, chronic kidney disease stage IV, and severe osteoporosis, whereas the older sister had normal stature (162 cm), one tiny hepatic nodule, and normal renal function. Direct sequencing of G6PC in two sisters identified a homozygous splicing mutation, c.645G>T, which is a prevalent mutation in Korea. Interestingly, our cases and four adults from recent reports had asymptomatic mild hypoglycemia and various manifestations including renal failure, HCC, fatty liver, or uncontrolled hyperlipidemia. These adult cases represent not only heterogenous phenotype to genotype within family members with GSD Ia but also long-term complications such as gouty arthritis, renal failure, and osteoporosis in untreated adult GSD Ia patients. In addition, lactic academia and hypertriglyceridemia are good markers of GSD Ia to distinguish from metabolic disease.

• Korean Journal of Clinical Laboratory Science
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• v.51 no.4
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• pp.468-474
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• 2019

Deep brain stimulation (DBS) is an effective surgical procedure for treating drug refractory movement disorders, and DBS involves delivering high frequency electrical stimulation to deep brain nuclei. Microelectrode recording (MER) is a complementary test that can precisely identify the location of deep brain nuclei, along with MRI correlation, during DBS surgery to improve the surgical outcome and minimize side effects. The purpose of this paper is to analyze the neuro-physiological waveforms and identify the usefulness of MER by analyzing the MER performed during DBS surgery for treating movement disorders. We retrospectively reviewed 28 patients who underwent MER during DBS surgery for movement disorders from January to December 2018. Of the 28 patients, 38 MERs for the subthalamic nucleus (STN), 10 MERs for the globuspallidusinternus (Gpi), and 4 MERs for the ventral intermediate thalamic nucleus (VIM) were performed. In all the cases, the target sites were found and micro-stimulations were used to check for side effects and to readjust the target sites. The clinical symptoms of all 28 patients improved after surgery. In conclusion, MER is a useful test that employs neuro-physiological waveforms to accurately identify the deep brain nuclei, along with MRI correlation, to improve the DBS surgical outcomes for movement disorders and to minimize side effects.

• Korean Journal of Psychosomatic Medicine
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• v.19 no.2
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• pp.74-82
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• 2011

Objective:The aim of this study was to evaluate the relationship between sustained attention deficits in Attention-Deficit/Hyperactivity Disorder(ADHD) children and short-term Heart Rate Variability(HRV) parameters. In addition, we evaluate the relationship between The ADHD rating scale(ARS), the computerized ADHD diagnostic system(ADS) and Parenting stress index- short form(PSI-SF). Methods:This study was performed in the department of children and Adolescent psychiatry, Korea university Guro hospital from august 2008 to January 2009. We evaluated HRV parameters by short-term recordings of 5 minutes. K-ARS and ADS are used for screening and identifying ADHD children. Intelligence was measured using Korean educational Developmental Institute-wechsler Intelligence Scale for Children. The caregivers Complete Parenting Stress Index scale for evaluation parent stress. Results:The low frequency(LF) was significantly correlated with response variability of ADS. However, the other variables of ARS and ADS were not significantly correlated with LF. Hyperactivity subscale of ARS was significantly correlated with parental distress subscale and difficult child subscale of PSI-SF and inattention subscale of ARS was also significantly correlated with dysfunctional interaction and difficult child subscale of PSI-SF. Conclusion:The LF, 0.10-Hz component of HRV is known to measure effort allocation. This study shows that the LF component of HRV is significantly correlated with the response variability of ADS. This means that more severe symptoms of ADHD were correlated with the increase in the LF that means decreased effort allocation. These results also support the clinical usability of HRV in the assessment of ADHD. Furthermore, PSI-SF is correlated with hyperactivity and inattention variables of ARS.

• Journal of Korean Society of Spine Surgery
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• v.25 no.4
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• pp.147-153
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• 2018

Study Design: Retrospective case series. Objectives: We retrospectively evaluated the clinical efficacy of postganglionic nerve block in symptomatic Schmorl nodules (SNs). Summary of Literature Review: SNs are common lesions that are often asymptomatic. In certain cases, SNs have been reported to cause severe axial back pain, thereby considerably impacting patients' quality of life. No consensus currently exists on the treatment of symptomatic SNs. Materials and Methods: From October 2015 to October 2017, a total of 21 patients with symptomatic SNs diagnosed by magnetic resonance imaging (MRI) that did not respond to conservative treatment after 4 weeks were included in the study. All patients received postganglionic nerve block. We evaluated effective pain relief (improvement of back pain of more than 50% compared with before the intervention) and functional improvements, assessed by visual analogue scale (VAS) and Oswestry Disability Index scores obtained at 4 hours, 4 weeks, 8 weeks, 3 months, and 6 months after the procedure. Results: Symptomatic SNs were more common at the L2-3 level, and the lower end plate was more commonly involved than the upper end plate. Eighteen of the 21 patients (85.7%) showed effective pain relief, and no deterioration was observed within the followup period. Throughout the follow-up period, the VAS remained significantly improved compared to before the procedure (p<0.05). Complications were not reported in any cases. Conclusions: Postganglionic nerve block for symptomatic SNs that do not respond to conservative treatment is a non-invasive modality for pain relief.