• Childhood Kidney Diseases
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• v.9 no.1
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• pp.31-37
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• 2005

Puruose : Thin glomerular basement membrane disease(TGBMD) is found in patients with family history of hematuria. TGBMD is autosomal dominant and is known to be one of the commonest causes of asymptomatic hematuria. This study was conducted to evaluate the histological and clinical features of patients with TGBMD. Methods : 150 cases diagnosed with TGBMD by renal biopsy while admitted in the department of pediatrics, Kyungpook National University Hospital between January 1999 and December 2003 comprised the study group. The following parameters were retrospectively anaIyzed age of onset, hematuria pattern, existence of proteinuria, process of diagnosis, laboratory findings, thickness and character of basement membrane and family history. Results : The mean age at the time of diagnosis was 7.9 years. The male to female ratio was 65:77. 94 patients or 66% visited the hospital with a chief complaint of persistent microscopic hematuria. Gross hematuria accounted for 13 cases or 9%. 78 cases(55%) were found to have hematuria for the first time from a routine school urinalysis screening. The renal biopsy showed the thickness of basement membrane to be 186$\pm$36 nm. Focal lamellation of the basement membrane was found in eight cases. In the family history, hematuria was shown in 10 cases on the Paternal side, 13 on The maternal side and none on both sides. In seven cases, hematuria was shown among siblings. No significant differences were found among the laboratory test results which were conducted at an average interval of fifteen months. Conclusion : TGBMD is one of the major causes of asymptomatic hematuria in children, which was diagnosed in increasing numbers since school urinary mass screening test started in 1998. In cases with familial progressive renal disease or focal duplication in the basement membrane Alport syndrome should be considered.

• Neonatal Medicine
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• v.18 no.1
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• pp.82-88
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• 2011

Purpose: This study aims to describe the clinical characteristics of adrenal insufficiency-associated hypotension in preterm infants and the effects of hydrocortisone therapy on their cardiovascular system and serum electrolytes. Methods: Twelve preterm infants less than 32 gestational weeks admitted to neonatal intensive care unit (NICU) of the Seoul National University Bundang Hospital from January 2007 to August 2009 with clinical and laboratory findings suggestive of adrenal insufficiency were analyzed retrospectively. Results: Gestational age was 27.8${\pm}$2.5 weeks and birth weight was 1,110${\pm}$307 g. Postnatal age, postmenstrual age, weight at the onset of adrenal insufficiency-associated hypotension were 19${\pm}$7 day, 30.6${\pm}$2.4 weeks, 1,285${\pm}$365 g. In preterm infants who showed vasopressor resistance, intravenous hydrocortisone was started with a stress dose of 4 mg/kg/day, maintained for 2.2${\pm}$0.7 days, and then tapered. Serum cortisol concentration before hydrocortisone administration was 11.6${\pm}$4.1 mg/dL. Mean blood pressure increased from 25.0${\pm}$5.4 mmHg to 35.0${\pm}$5.3 mmHg, 38.3${\pm}$8.0 mmHg and 41.9${\pm}$6.5 mmHg at time of hydrocortisone administration and 2, 4 and 6 hours after hydrocortisone administration. Urine output increased from 0.9${\pm}$0.6 mL/kg/hr to 4.1${\pm}$3.4 mL/kg/hr. Twelve hours after the administration of hydrocortisone, dopamine requirement decreased from 11.0${\pm}$2.9 $\mu$g/kg/min to 8.0${\pm}$2.3 $\mu$g/kg/min, and to 5.5${\pm}$3.4 ${\mu}g$/kg/min after 24 hours. Serum sodium concentration was increased from 130${\pm}$4 mEq/L to 136${\pm}$4 mEq/L, serum potassium concentration was decreased from 6.1${\pm}$1.1 mEq/L to 4.6${\pm}$0.6 mEq/L before and 12 hours after hydrocortisone administration. Conclusion: In preterm infants with adrenal insufficiency-associated hypotension, hydrocortisone administration improved blood pressure and urine output, decreased vasopressor requirement, and normalized serum electrolyte abnormalities.

• Pediatric Infection and Vaccine
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• v.9 no.2
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• pp.163-174
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• 2002

Background : To delineate the changes in the causative agents of neonatal sepsis and their antimicrobial susceptibilities in the neonatal intensive care unit and nursery of Hanyang University Hospital during the past 10 years. Methods : Hospital records of 15,144 patients hospitalized at the NICU and nursery of Hanyang University Hospital from 1989 to 1998 were reviewed and neonates diagnosed of neonatal sepsis were sorted and included in the study. The study period was divided into Period A(the first 5 years) and Period B(the second 5 years) to analyse causative agents and their antimicrobial susceptibilities. Results : Neonatal sepsis was diagnosed in 170 patients(1.1%{Period A 1.2%, Period B 1.0%}) among the total of 15,144 inpatients. Two hundred isolates(Period A 109 isolates, Period B 91 isolates) were identified in 186 blood cultures(Period A 99 cultures, Period B 87 cultures) from 170 patients(Period A 91 patients, Period B 79 patients). The average age at the onset of the disease, when the initial blood culture was drawn, was 12.3 days old(Period A 8.8 days, Period B 16.3 days), and the proportion of the early onset disease was 34.7% in Period A and 23.0% in Period B, indicating that neonatal sepsis developed earlier during Period A. Among the isolated organisms including Gram positive bacteria[132(66.0%)], Gram negative bacteria [60(30.0%)], and fungi[8(4.0%)], coagulase negative Staphylococcus(CNS) was the most common organism(69/34.5%), followed by Staphylococcus aureus(36/18.0%), Klebsiella pneumoniae(17/8.5%), Enterococcus(12/6.0%), Enterobacter cloacae(8/4.0%), Escherichia coli(6/3.0%), and Pseudomonas aeruginosae(5/2.5%). The isolated fungi were Candida parapsilosis, Candida albicans, and Trichosporon pullulans. CNS, S. aureus and Acinetobacter baumannii were isolated more frequently in Period A compared to Period B. Antimicrobial susceptibilities of CNS and S. aureus to methicillin and the first generation cephalo sporins were decreased in Period B compare to Period A, those to aminoglycosides were increased in Period B, and vancomycin resistant strains were not identified. K. pneumoniae, Enterococcus, E. coli, and P. aeruginosa were isolated less frequently in Period B, compared to Period A. For K. pneumoniae, antimicrobial susceptibilities to the first generation cephalosporins were low in both Periods A and B, those to tobramycin and gentamicin were increased in Period B, and those to amikacin, ceftriaxone, and trimethoprim-sulfamethoxazole were high in both Periods A and B. Antimicrobial susceptibilities of Enterococcous to ampicillin, penicillin, and the first generation cephalosporins were decreased in Period B, but vancomycin resistant strains were not identified. Conclusion : The occurrence rate of neonatal sepsis during the past 10 years in the NICU and nursery of the Hanyang University hospital was 1.1%, and the most common causitive agents were CNS and S. aureus, to which the antimicrobial susceptibilities to the first line drugs decreased in the later half of the study period with no vancomycin resistant isolates identified. Group B Streptococcus known to be the most common agent causing neonatal sepsis was not identified, and K. pneumoniae was isolated more commonly during the later half of the study period without decreased antimicrobial susceptibilities.

• Pediatric Infection and Vaccine
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• v.14 no.1
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• pp.43-46
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• 2007

Purpose : Neonatal bacterial meningitis has been known that its mortality and morbidity is high and its neurologic complications are common. This study was performed to review of the etiology of neonatal bacterial meningitis in Busan, Korea. Methods : We retrospectively analyzed 21 medical records of patients who were diagnosed neonatal bacterial meningitis in four university hospitals in Busan from January 1997 to December 2005. We analyzed age at onset of symptoms, symptoms at admission, causative organisms, and complications. Results : There were 11 male patients and 10 female patients. One premature neonate was included. 7 patients had symptoms at 2-3 weeks after birth and 6 patients at 1-2 weeks after birth. 18 cases had fever, 8 cases had seizure and some had poor feeding, lethargy and irritability. The most common organism causing neonatal bacterial meningitis was Group B streptococcus (GBS). 11 cases were due to GBS, 3 cases due to E. coli, 3 cases were due to Klebsiella pneumoniae, and 2 cases due to Streptococcus pneumoniae. one Listeria monocytogenes and one Enterobacter cloacae were included. 18 patients were alive and 3 patients died. Mental retardation, developmental delay and epilepsy occurred in a living patient. Conclusions : Although this was a retrospective study, we found that Group B streptococcus (GBS) was the most common organism causing neonatal bacterial meningitis and this data is nearly same as others in the world.

• Pediatric Infection and Vaccine
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• v.26 no.3
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• pp.179-187
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• 2019

Purpose: Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome is a leading cause of periodic fever in children. This study describes the clinical characteristics of PFAPA syndrome in patients from a single center. Methods: Thirteen children diagnosed with PFAPA syndrome at Seoul National University Children's Hospital were included in this study. Retrospective medical chart reviews were performed. Results: Among the 13 patients, 8 (61.5%) were male. The median follow-up duration was 3.3 years (range, 10 months-8.3 years). The median age of periodic fever onset was 3 years (range, 1-6 years). All patients had at least 5 episodes of periodic fever and pharyngitis, managed with oral antibiotics, before diagnosis. The median occurrence of fever was every 3.9 weeks and lasted for 4.2 days. All patients had pharyngitis and 12 (92.3%) had cervical lymphadenitis. Blood tests were performed for 12 patients, and no patients had neutropenia. Both the C-reactive protein and erythrocyte sedimentation rate were elevated at medians of 4.5 mg/dL (range, 0.4-13.2 mg/dL) and 29 mm/hr (range, 16-49 mm/hr), respectively. Throat swab cultures and rapid streptococcal antigen tests were negative. Nine (69.2%) patients received oral prednisolone at a median dose of 0.8 mg/kg, and in 6 (66.7%) patients, fever resolved within a few hours. Three (23.1%) patients received tonsillectomy and adenoidectomy. Conclusions: PFAPA syndrome should be considered when a child presents with periodic fever along with aphthous stomatitis, pharyngitis, or cervical lymphadenitis. Glucocorticoid administration is effective for fever resolution and can reduce unnecessary use of antibiotics.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.5 no.1
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• pp.33-43
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• 2005

Purpose: X-linked adrenoleukodystrophy(ALD) is a rare disorder that shows a great deal of phenotypic variability. We subdivided chidhood X-linked ALD patients into several phenotypes by the age at onset, the sites of most severe clinical involvement and the rate of progression of neurologic symptoms. Methods: Thirteen patients who had been diagnosed as X-linked ALD and followed up for at least one year were enrolled from 1996 to 2003. Results: 1. Ten had childhood cerebral ALD, who showed first neurologic symptoms at 7.02 years and progressed rapidly: interval between first symptoms and vegetative state was 1.35 years, and interval from initial symptoms to death was 3.35 years. Treatment with Lorenzo's oil did not prevent neurologic progression. Two patients who underwent umbilical cord blood transplantation died. 2. Two had adolescent cerebral ALD. They had first symptoms at 11.5 years, and showed tendency to progress less rapidly than childhood cerebral form patients. 3. One "Addison only" patient who had adrenal insufficiency without nervous system involvement remained asymptomatic during Lorenzo's oil treatment. 4. All cerebral form patients except one showed the lesions in both parieto-ocipital white matter in brain magnetic resonance imaging. Conclusion: The cerebral ALD was the most common form in childhood and was asoociated with a serious prognosis.

• Childhood Kidney Diseases
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• v.2 no.1
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• pp.14-19
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• 1998

Purpose : Hypocalcemia is one of the major causes of convulsion in infants. The causes of hypocalcemia are hypoparathyroidism, deficiency and metabolic abnormalities of vitamin D, or increased uptake of inorganic phosphorous, etc. We analyzed the causes, symptoms and signs, treatment, and clinical courses of hypocalcemia as, recently, there were many clinical experiences of hypocalcemic infants under age of 6 months in the department of pediatrics, Kyungpook University Hospital. Objects and Methods : The authors observed 11 infants with hypocalcemia who had been admitted to the department of pediatrics, Kyungpook University Hospital, during the period of February 1992 to April 1997. Various clinical and laboratory data concerning causes, clinical courses and treatment of hypocalcemia were analyzed retrospectively Results : (1) The sex incidence revealed male predominance with male to female ratio 4.5 : 1. The mean age at onset of symptoms was $2.2{\pm}1.1$ months old. (2) The causes of hypocalcemia were vitamin D deficiency in 8 cases and excessive inorganic phosphate intake in 3 cases. (3) All eleven patients manifestated convulsion which was generalized tonic-clonic in 9, and focal clonic in 2 cases. (4) Serum calcium concentrarion increased from $6.3{\pm}0.9$ mg/dL to $9.9{\pm}1.7$ mg/dL after therapy of $1,25(OH)_{2}D_{3}$ with or without calcium(P=0.0008), and serum ALP concentration decreased from $1,418{\pm}864$ U/L to $772{\pm}503$ U/L (P=0.0112). Serum iPTH levels were high in all 11 patients initially. All showed decreased $25(OH)D_3$ levels initially. (5) All patients were treated successfully with $1,25(OH)_{2}D_{3}$ and/or calcium supplement. Conclusions : Vitamin D deficiency should be considered as one of the causes of hypocalcemia even in formula(known as vitamin fortified) feeding infants. Fortunately, they were successfully treated with $1,25(OH)_{2}D_{3}$.

• Neonatal Medicine
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• v.18 no.2
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• pp.197-203
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• 2011

Purpose: The purpose of this study is to compare perinatal outcomes between in vitro fertilization (IVF) twins and naturally conceived twins born to women aged 35 years or older and to provide basic information for taking care of IVF twins born to women aged 35 years or older. Methods: We reviewed the records of perinatal and neonatal outcomes in 288 IVF twins and 220 naturally conceived twins born to women aged 35 years or older between January 2001 and December 2010 at CHA Bundang Medical Center. Results: No difference was observed in the maternal ages of mothers giving birth to IVF twins and those giving birth to naturally conceived twins. Gestational ages and birth weights of IVF twins were not different from those of naturally conceived twins. Various perinatal outcomes, including gestational diabetes mellitus, pregnancy-induced hypertension, placenta previa, premature amniotic membrane rupture, and need for a Cesarean section did not differ between the 2 groups. However, the 1-min and 5-min Apgar scores (P=0.019 and P=0.045, respectively) were different between the 2 groups. The incidence of early-onset sepsis was lower in the IVF twins than in the naturally conceived twins (P=0.02). However, the 2 groups did not show any difference in the incidence of respiratory distress syndrome, bronchopulmonary dysplasia, patent ductus arteriosus, necrotizing enterocolitis, intraventricular hemorrhage, and other congenital anomalies. Conclusion: The perinatal outcomes in IVF twins born to women aged 35 years or older were not significantly different from those of naturally conceived twins.

• Tuberculosis and Respiratory Diseases
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• v.42 no.2
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• pp.175-183
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• 1995

Background: The incidence of mycoplasmal pneumonia is predominantly at childhood and early adulthood, but in adults, its incidence is low and its symptoms and physical findings are nonspecific. The definite diagnosis of M. pneumoniae pneumonia can be made by sputum culture, but requires several weeks for positive results, and the early diagnosis must initially be based on the serologic tests and appropriate clinical findings. Thus, we evaluated the clinical aspects of M. pneumoniae pneumonia in the adults patients. Method: Among the admitted patients due to pneumonia, the definite diagnosis is anti-M. pneumoniae antibody titer of $\geq$ 1:40 and a single cold agglutinin titer of $\geq$ 1:64. The presumptive diagnosis is anti-M. pneumoniae antibody titer of $\geq$ 1:40 or a single cold agglutinin titer of $\geq$ 1:64 and the clinical characteristics or chest X-ray findings are compatible with M. pneumoniae pneumonia. We studied the age and sex distribution, seasonal distribution, clinical symptoms, physical findings, serologic test, chest X-ray findings, treatment and its progression. Results: 1) The age distribution was even and the ratio of male to female was 1:1. 2) The monthly distribution was most common in January(16.7%) and the seasonal distribution in autumn and winter(autumn: 30%, winter: 33.3%). 3) The cold agglutinin titers were higher than 1:64 in 12 cases(40%), and reached the peak level around 2 weeks from onset and antimycoplasma antibody titers were higher than 1:160 in 5 cases(16.7%). 4) On the chest X-ray, pulmonary infiltration was noted in 28 cases(93.3%) among 30 cases and right lower lobe involvement was the most common(33.3%) and both lower lobe involvement was noted in 7 cases(23.3%). 5) The mean treatment duration was most common(33.3%) in 1 week to 2 weeks after admission and 26 cases(86.7%) were improved within 4 weeks. 6) On admission, there was fever(${\geq}38.9^{\circ}C$) in 17 cases(56.7%), and the fever subsided in 12 cases(70%) within 3 days after treatment using erythromycin. Conclusion: The mycoplasmal pneumonia in adults shows milder clinical patterns than that in childhood and can be completely recovered without complication by early diagnosis and treatment.

• Journal of Korean Orthopaedic Sports Medicine
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• v.4 no.2
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• pp.122-127
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• 2005

Purpose: To study the mechanism of injuries or discomfort during kendo and to analyze the causes of injury, injured parts of body, treatments modalities, and degree of return to the pre-injury activity level. Materials and Methods: We studied ninety-eight kendo players who had played at three gymnasium of Korean Kumdo Association, were older than high-school age, had been playing kendo for more than six months, and had any pain or discomfort after playing kendo. We analyzed the questionnaire and interviewed the patients concerning the injured parts of body, symptoms, treatments. Results; The mean age was 30.2 years old, and the onset of symptoms was between two weeks and six months after starting the kendo. Many of them were injured during attacking(32.2%). The injuries of feet were increased on players who exercised for less than 1 year. Only fifty-nine players(60.2%) were treated within one month. Among them, thirty-eight percent were treated by oriental medicine or other departments and only thirty-six percent were treated by orthopaedic surgeons. The prevalence of injuries of the foot and ankle was highest(38.8%), and there were the wrist(13.1%), shoulder(11.9%), knee(11.9%) in order of prevalence. Fifty percents of them could return to the pre-injury activity level. Conclusion: The prevalence of injuries of the foot and ankle was highest(38.8%), and were increased on players who exercised for less than 1 year. Sixty percents of players were treated with any proper modalities, and only thirty-six percent were treated by orthopaedic surgeons.