• Childhood Kidney Diseases
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• v.8 no.2
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• pp.176-185
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• 2004

Purpose: Rapidly progressive glomerulonephritis (RPGN) is a clinicopathologic entity characterized by extensive crescent formation and rapid deterioration of renal function within few months. For better understanding of its clinical course and designing better treatment strategies, a clinicopathological study of childhood RPGN was performed. Methods: The clinical manifestations and pathological findings were reviewed retrospectively in 12 children who were diagnosed as having RPGN by clinical manifestations and renal biopsy during a period from 1991 to 2003. Several clinicopathological parameters were analyzed as prognostic factors. Results: Among a total of 12 patients, 4 were male and 8 were female. The median onset age was 11.5 years(range 5.5-14.6 years), and the median period of follow-up was 25 months(range 7 months-6.6 years). According to the pathological classification, 10 patients (83%) were type II RPGN(immune-complex mediated glomerulonephritis), 2 patients were type III RPGN(pauci-immune glomerulonephritis), and none was type I RPGN(anti-glomerular basement membrane nephritis). All patients were treated with oral steroid in various combinations with methylprednisolone pulse therapy(10 patients, 83%), cyclophosphamide(8 patients, 67%), or plasmapheresis(4 patients, 33%). Clinical outcomes of 12 patients were complete remission in 1(8%), end-stage renal disease in 2(17%), chronic renal insufficiency with persistent proteinuria in 2(17%), and normal renal function with persistent proteinuria in 7(58%) at the last follow-up. Poor prognosis is associated with increased serum creatinine level, severe anemia and younger age at the time of diagnosis. Conclusion: Immune-complex mediated glomerulonephritis is the major cause RPGN in children and most cases showed improvement of renal function with aggressive management. For better understanding of this rare disease, a prospective multicenter study should be done.

• Journal of Chest Surgery
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• v.40 no.5 s.274
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• pp.351-355
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• 2007

Background: Postinfarction ventricular septal rupture is associated with mortality as high as $85\sim90%$, if it is treated medically. This report documents our experience with postinfarction ventricular septal rupture that was treated surgically, Material and Method: We retrospectively reviewed the medical records of 11 patients who were operated on due to postinfarction ventricular septal rupture between August 1996 and August 2006. There were 4 men and 7 women, with a mean age of $70{\pm}11$ years (age range: $50\sim84$ years). The location of the rupture was anterior in 7 cases and posterior in 4 cases. The interval between the onset of acute myocardial infarction and the occurrence of the ventricular septal rupture was $2.0{\pm}1.3$ days (range: $1\sim5$ days). Operation was performed at an average of $2.4{\pm}2.7$ days (range: $0\sim8$ days) after the diagnosis of septal rupture. Preoperative intraaortic balloon pump therapy was performed in 10 patients. Result: The infarct exclusion technique was used in all cases. Coronary artery bypass grafting was done in 8 cases, with the mean number of distal anastomosis being $1.0{\pm}0.8$. There was one operative death. In 2 patients, reoperation was performed due to a residual septal defect. The postoperative morbidities were transient atrial fibrillation (n=7), paroxysmal supraventricular tachycardia (n=1), low cardiac output syndrome (n=3), bleeding reoperation (n=2), delayed sternal closure (n=2), acute renal failure (n=2), pneumonia (n=1), intraaortic balloon pump-related thromboembolism (n=1), and transient delirium (n=2). Nine patients have been followed up for a mean of $38{\pm}40$ months except for one follow-up loss. There have been 3 late deaths. At the latest follow-up, all 6 survivors were in a good functional class. Conclusion: We demonstrated satisfactory operative and midterm results with our strategy of preoperative intraaortic balloon pump therapy, early repair of septal rupture by infarct exclusion and combined coronary revascularization.

• Journal of the korean academy of Pediatric Dentistry
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• v.33 no.3
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• pp.457-468
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• 2006

It is widely known that individuals with mental retardation (MR) and Down's syndrome (DS) often develop early onset periodontal diseases. In this study, the prevalence of periodontopathic bacteria in MR persons and DS patients was compared with normal persons. Plaque index and gingival index were measured. Five periodontopathic bacteria, Actinobacillus actinomycetemcomitans. Porphyromonas gingivalis, Tannerella forsythia, Treponema denticola, Fusobacterium nucleatum were surveyed in subgingival plaque samples by the polymerase chain reaction. Results : 1. Plaque index and gingival index were higher in MR persons group and DS patients group than normal persons group (p<0.05). 2. The prevalence of periodontopathic bacteria in normal persons group were lower than that of MR persons group and DS. Significant differences were observed in the prevalence of P. gingivalis, T. denticola and A. actinomycetemcomitans(P<0.05). 3. Prevalence of P. gingivalis(5.9%) at age 8-10 was lower than other ages in normal persons group, and its prevalence increased with age Prevalence of P. gingivalis, T. denticola and A. actinomycetemcomitans at MR persons group and DS patients group were higher than those of same ages of normal persons group. 4. Plaque index was associated with T. denticola and gingival index was associated with T. denticola and A. actinomycetemcomitans(P<0.05). These results suggested that plaque index, gingival index and prevalence of periodontopathic pathogens, especially P. gingivalis, T. denticola and A actinomycetemcomitans in DS patients group and MR persons group are higher than those of normal persons group.

• Clinical and Experimental Pediatrics
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• v.52 no.5
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• pp.538-543
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• 2009

Purpose : To report the clinical experience with amitriptyline for managing children with cyclic vomiting syndrome (CVS). Methods : Totally, eleven children (eight males) were diagnosed with CVS. Of these, medical records were reviewed for eight children treated with amitriptyline; three children were not treated because one was not followed up and two were kept under observation to study the natural course without medication, because the frequency of vomiting had recently decreased to less than one episode per year. The eight amitriptyline-treated children were divided into three groups by the therapeutic response: (1) complete remission (CR), with no recurrence of symptoms after treatment; 2) partial remission (PR), in which the frequency of symptoms decreased by almost 50% after treatment; and (3) no response. Results : For the eight amitriptyline-treated children, the mean age of symptom onset was 4.7 (0.3-7) yr and mean age at diagnosis was 8.9 (6.0-11.3) yr. The mean number of vomiting episodes was 8.8 (2-25) per year, and the duration of vomiting in each episode ranged from 3 hours to 5 days. CR was achieved in five (62.5%) of the eight amitriptyline-treated children (0.2-0.8 mg/kg/day orally, at night) and PR was achieved in three children (37.5%). Two children relapsed on discontinuation of amitriptyline by their parents decision but showed improvement on remedication. No adverse effects were noted throughout the study period. Conclusion : Amitriptyline can be used in long-term prophylaxis for the management of children with CVS aged over 6 yr. However, a large-scale study is needed to ascertain its effects.

• 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.