• Childhood Kidney Diseases
• /
• v.6 no.1
• /
• pp.48-55
• /
• 2002

Purpose: Thin glomerular basement membrane nephropathy (TGBMN) is recognized as the leading cause of microscopic hematuria in both children and adults. However thinning of glomerular basement membrane (TCBM) has been found in healthy adult and also is known to be associated with various renal diseases such as Alport syndronh, IgA nephropathy and mesangial proliferative glomerulonephritis. The association of TGBM with minimal change nephrotic syndrome (MCNS) has been very rare so that the present study was undertaken to determine the relationship between TGBM and MCNS. Methods: The study population consisted of 49 children with biopsy- proven MCNS who have been admitted to the pediatric department of Kyungpook University Hospital during the past 5 years from 1997 to 2001. Group I consisted of 8 children associated with TGBM and Group II 41 children without TCBM. Various parameters such as age of illness, duration from discovery of illness to the time of biopsy, family history of hematuria and other laboratory tests were compared between these two groups and the following results were obtained. Results: Age distribution showed slightly older age in Group I ($7.1{\pm}3.5$ years) compared to Group II ($4.8{\pm}2.9$ years). However this was not statistically different (P=0.056). Family history of hematuria was noted in 2 cases in Group II. Though statistically not significant, hematuria was seen in 2 out of 8 cases ($25\%$) in MCNS children with TGBM, compared to 7 out of 41 cases ($17\%$) with MCNS children without TGBM. Other parameters such as BUN, creatinine, 24 hours urine protein excretion, serum protein, albumin, cholesterol, and T4/T8 ratio, showed no difference. Also renal biopsy finding showed no significant difference and the thickness of glomerular basement membrane in Croup I was $188{\pm}30nm$. Conclusion: TGBM was found in 8 out of 49 children with MCNS ($16.3\%$). And this high frequency of occurrence indicates that these association is not an incidental findings. Typical clinical findings of TCBMN was not noted in all of the 8 children with MCNS associated with TGBM, suggesting that thinning of glomerular basement membrane (TCBN) is secondary to rather than the cause of MCNS. (J Korean Soc Pediatr Nephrol 2002;6: 48-55)

• Childhood Kidney Diseases
• /
• v.6 no.2
• /
• pp.178-187
• /
• 2002

Purpose : The detection of hydronephrosis(HN) with antenatal ultrasonography was first reported in the 1970s. Prenatal HN is diagnosed with an incidence of 1:100 to 1:500 on antenatal screening. Recently, the purpose of antenatal screening has changed from simple detection to selection for specific diagnosis-based management. this study is to evaluate the usefulness of antenatal sonography for HN and to investigate the differential causes of HN and their clinical outcomes. Patients and methods : 11,783 live neonates with prenatal ultrasonographic examination at Ajou University School of Medicine, from Sep. 1994 to Aug. 2001 were analyzed. Results and conclusion : Hydronephrosis (>10 mm) was detected in 119 (1.0%) cases antenatally and among these, 91 were proved to have HN postnatally Males were three times more affected than females. Additional imaging studies revealed that ureteropelvic junction obstruction was the most common postnatal diagnosis (47%), followed by multicystic dysplastic kidney, vesicoureteral junction obstruction and vesicoureteral reflux. During 20 months' follow-up(3 to 72 months), 58(48%) renal units showed spontaneous resolution and surgical interventions were necessary in 10 (7.4%) of postnatally confirmed hydronephrotic renal units.

• Journal of Chest Surgery
• /
• v.35 no.1
• /
• pp.1-10
• /
• 2002

Background: Despite the relatively high mortality rates in the chronic heart failure model induced by coronary artery ligation are relatively high, this model has been a subject of continuos research because of its clinical correlation. Chronic heart failure model of large-sized animals is very useful to analyse mechanical or biological effects on circulatory system which is difficult in small-sized animals. The purpose of this study is to establish the heart failure model by coronary artery ligation in sheep. Material and Method: Among 9 Corridale sheep, the homonymous artery and the diagonal branch were ligated simultaneously in 2 sheep and remaining 7 sheep were assigned to successive ligation of both arteries at an interval of 1 hour. Both coronary arteries were ligated from the point 40% proximal to the apex of the heart. Hemodynamic and echocardiographic parameters were analyzed before the ligation of the coronary artery, after the ligation of the homonymous artery, and after additional ligation of the diagonal branch. The experimental animals were sacrificed after 2 or 3 months of growth and histopathologic studies were performed Result: Immediate postoperative death occurred in the 2 sheep that had received simultaneous ligation of the homonymous artery and diagonal branch. On the other hand, all the 7 sheep that were lifated in succession were survived up to 3 months. Arterial pressure was sifnificantly decreased immediately after ligation of the homonymous artery(p<0.05), and the cardiac output was decreased and pulmonary capillary wedge pressure was increased after further ligation of the diagonal branch(p<0.05). Central venous pressure, pulmonary artery pressure, pulmonary capillary wedge pressure, left ventricular end-diastolic dimension and end-systolic dimension were markedly increased 3 months after ligation of coronary arteries. Anteroseptal akinesia or dyskinesia was developed after the ligation of coronary arteries. Histopathologic study revealed we]1-demarcated ischemic area of fibrosis. Conclusion: Using methods of successive ligation of the homonymous artery and diagonal branch, chronic heart failure model could be reliably established in sheep.

• Clinical and Experimental Pediatrics
• /
• v.46 no.12
• /
• pp.1248-1252
• /
• 2003

Purpose : Chest pain in the pediatric population is not rare and mostly benign. Causes of chest pain are diverse, and differential diagnosis is not easy. Chest pain in children is less likely to be cardiac in origin. Furthermore, chest pain in the pediatric population is rarely associated with life-threatening disease. This study was designed to evaluate children with chest pain and the usefulness of several diagnostic examinations. Methods : Between March 2001 and August 2002, 33 patients(15 boys and 18 girls, aged four to 15 years) presented with chronic chest pain. The records of these patients were reviewed. Chest radiography and electrocardiogram were performed in all patients. Cardiologic and gastrointestinal evaluations were carried out when considered necessary. Results : Chest pain was most common in the age group of 10 to 12 years old, and the four to six years old group. The most common diagnostic findings of chest pain were idiopathic(15 cases, 45.5 %), heart disease(9 cases, 27.3%), upper gastrointestinal disease(6 cases, 18.2%), respiratory disease (2 cases, 6%) and trauma(1 case, 3%). In children with abnormal results of cardiologic evaluation, these findings are not major etiologic categories of chest pain. Through history taking and physical examinations, six cases were evaluated concerning gastrointestinal disease and all of them showed gastrointestinal diseases(esophagitis, gastroesophageal reflux disease, nodular gastritis and chronic superficial gastritis). Conclusion : Chest pain is usually benign in children but the possibility of cardiovascular or gastrointestinal disease is considered. Careful history taking, physical examination and proper clinical examinations are usually required to find out the rare life-threatening causes of chest pain.

• Journal of Nutrition and Health
• /
• v.47 no.2
• /
• pp.113-123
• /
• 2014

Purpose: Vitamin D has been known to play an important role in the central nervous system and brain functions in the human body, and cumulative evidence has shown that vitamin D deficiency might be linked with various mental health conditions. Epidemiologic studies have shown that vitamin D deficiency may be associated with higher risk of depression in the US and European populations. However, limited information is available regarding the association between vitamin D status and depression in the Korean population. The objective of this study was to examine the associations between vitamin D levels and prevalence of depression. Methods: We conducted a cross-sectional analysis using nationally representative data from the 2008-2010 Korean National Health and Nutrition Examination Survey from which serum 25-hydroxyvitamin D concentrations were available. A total of 18,735 adults who had available demographic, dietary, and lifestyle information were included in our analysis. We defined "depression" with a diagnosis by a physician. "Depressed condition" was defined as having feelings of sadness or depression without diagnosis by a physician. Results: The prevalence of depression was 1.63% and 5.43% in Korean men and women, respectively; 12.5% of men and 26.1% of women were defined as the group having depressed conditions. In multivariate logistic regression models, no significant associations were observed between vitamin D status and prevalence of depression or depressed conditions in Korean men and women. Conclusion: We found no association between vitamin D insufficiency and depression/depressed conditions in Korean adults. Future large prospective studies and randomized controlled trials are needed to confirm this relationship.

• Journal of Gastric Cancer
• /
• v.8 no.3
• /
• pp.129-135
• /
• 2008

Purpose: The usefulness of serum tumor markers for assessing gastric carcinoma is very limited compared to that for neoplasms in other digestive organs. Many reports have shown that serum tumor markers are closely associated with the prognosis and tumor recurrence in gastric cancer patients. However, little is known about the usefulness of serum tumor markers as a predictor of distant metastasis for gastric carcinoma. Materials and Methods: With excluding the non-specific causes of elevated tumor markers, a total of 788 patients with gastric carcinoma and who were seen at our hospitals between 2004 and 2006 were included in this study. The correlation between the preoperative level of tumor makers and the clinicopathological features was analyzed. Results: CEA was significantly correlated with age, gender and nodal metastasis, but not with the depth of tumor. The CEA level was not correlated with distant metastasis, such as peritoneal or hematogenous metastasis. In contrast, the CA 19-9 level was significantly correlated not only with the depth of tumor and nodal metastasis, but also with peritoneal metastasis. Especially, the patients with over 500% elevation of the CA 19-9 level had a significant risk of peritoneal metastasis. Conclusion: CA 19-9 is useful for predicting peritoneal metastasis in gastric cancer patients. It can be used efficiently in making the diagnostic and the treatment plan, in combination with other diagnostic tools, for gastric cancer patients.

• Childhood Kidney Diseases
• /
• v.7 no.1
• /
• pp.23-29
• /
• 2003

Purpose : This study was performed to determine the natural history of histologically confirmed IgA nephropathy in pediatric patients who presented with hematuria and proteinuria. Patients and Methods : We reviewed the clinical course of 57 patients diagnosed with IgA nephropathy at the age of 15 years or younger from 1981 to 2000. All patients presented with hematuria or minimal proteinuria($<40\;mg/m^2/day$) and had normal renal function and blood pressure at the time of renal biopsy. Based on the clinical and pathological findings at the time of diagnosis, we sought for complications of IgA nephropathy such as heavy proteinuria(${\ge}40\;mg/m^2/day$), hypertension, and chronic renal failure. Results : The mean age at presentation was $9.5{\pm}2.8$ years(4 to 15 years) and 42(74%) were male. Isolated gross hematuria was observed in 20 patients(35%), microscopic hematuria in 3(5%), minimal proteinuria in 4(7%), both gross hematuria and minimal proteinuria in 15(26%), and both microscopic hematuria and minimal proteinuria in 15(26%). During a median follow-up of $7.0{\pm}3.5$ years, 38(67%) had complete resolution of hematuria and proteinuria, 12(21%) had persistently abnormal urinalysis without development of adverse events. Only 7(12%) developed adverse events : 4(7%) developed severe proteinuria, 1(2%) became hypertensive, and 2(3%) developed Impaired renal function. By univariate analysis using the chisquare test, the age at presentation(>10 years)(P<0.01) and poor histological classes of the Lee or Haas classification at onset(P<0.05) were significantly correlated with adverse events, whereas sex and clinical signs at onset were less concordant. Conclusion : We can conclude that the prognosis of IgA nephropathy diagnosed in early childhood is better and a good correlation exists between the clinical manifestations of this disease and the histological classes.

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

• Childhood Kidney Diseases
• /
• v.9 no.1
• /
• pp.21-30
• /
• 2005

Purpose : Metabolic bone diseases have been mai or problems in children with renal diseases and steroid treatment is the main precipitating factor reducing bone mineral density(BMD). This study was performed to assess the prevalence of osteoporosis and to evaluate the clinical factors associated with decreased BMD in children with renal diseases. Methods : Forty-four children with renal diseases who were diagnosed at the Pediatric no phrology division of Ajou University hospital since Oct. 1994 were included. Using a new quantitative ultrasound device, BMD and the prevalence of osteoporosis were evaluated. The clinical and serological data were analyzed in association with decreased BMD. Results : A total of 44 patients were evaluated. The age at initial diagnosis was 6.7$\pm$4.2 years. At the time of evaluation, the chronological and bone age was 9.3$\pm$4.2 years and 8.2 $\pm$ 4.6 years, respectively. The renal diseases included nephrotic syndrome 24(54.5%), Henoch Schonlein purpura nephritis 7(15.9%), IgA nephropathy 6(13.9%), reflux nephropathy(RN) 2 (4.5%), and other renal disease 5(%). The prevalence of osteoporosis was 11%. There was no difference in the clinical factors between the long-term and the short-term treated steroid groups. Conclusion : The prevalence of osteoporosis was 12% in 44 children with renal diseases No significant factor was found in association with decreased BMD and there was no relationship between osteoporosis and steroid usage duration or cumulative dose. A new quantitative ultrasound, which is relatively easy to perform, especially in children, is expected to be in common use and will enable clinicians to evaluate metabolic bone disorders with ease.

• Korean Journal of Clinical Laboratory Science
• /
• v.48 no.3
• /
• pp.255-261
• /
• 2016

The purpose of this study was to investigate the relationship between excessive daytime sleepiness (EDS) and blood pressure (BP) in patients with obstructive sleep apnea-hypopnea (OSAH). Patients were classified into four groups based on their severity of polysomnographic data: the snoring group (n=108)-characterized by Apnea-Hypopnea Index (AHI<5); the mild OSA group (n=186)-AHI $5{\leq}AHI$<15; the moderate OSA group (n=179)- AHI $15{\leq}AHI$<30; and the severe OSA group (n=233)-$AHI{\geq}30$. On the same night of polysomnography (PSG), BP levels were measured before sleeping (bedtime BP) and immediately after waking up on the following morning (morning BP). EDS was recognized as ESS (epworth sleepiness scale)${\geq}9$. The differences and correlations between BP and PSG parameters in the EDS and non-EDS groups of OSAH patients were analyzed. MAP was positively correlated with BMI, AHI, and total arousal (r=0.099, r=0.142, r=0.135, p<0.01, p<0.01, p<0.01), while negatively correlated with mean $SaO_2$ (r=-0.258, p<0.01). The EDS group had overall younger population ($47.2{\pm}11.3$ vs $50.3{\pm}11.4$, p=0.023), higher DBP (both bedtime and morning, $83.1{\pm}9.7$ vs $81.4{\pm}8.8$ and $86.4{\pm}9.2$ vs $83.6{\pm}9.7$)(p=0.031, p=0.047), and higher SBP (both bedtime and morning, $126.7{\pm}11.2$ vs $123.4{\pm}12.4$, $128.9{\pm}12.4$ vs $125.3{\pm}12.9$)(p=0.021, p=0.021) than compared with the non-EDS group. In hypertensive OSAH patients, patients with EDS were also younger and had higher total arousal number, as well as higher morning and bedtime DBP and SBP than compared with the non-EDS group (p<0.005, p=0.008, p<0.001 and p<0.001). EDS in OSAHS patients is a special phenotype characterized by younger age, higher DBP, more severe desaturation, and hypertension.