• Journal of Korean Neurosurgical Society
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• v.29 no.5
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• pp.644-649
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• 2000

Objective : Acute hydrocephalus can be caused by many pathologic conditions such as sub- arachnoid hemorrhage, intraventricular hemorrhage, inflammatory diseases. External ventricular drainage(EVD) through trephination of the skull is essential procedure for progressing or persistent symptomatic acute hydrocephalus. If the EVD can not be removed in short period, the chance of ventriculitis increases and periodic transposition of the draining catheter should be considered. Shunt procedure can not be performed in acute hemorrhage or infectious condition because of the risk of shunt malfunction or intra-abdominal spreading of the infection, respectively. The authors replaced EVD with continuous lumbar drainage(CLD) for the purpose of controlling acute hydrocephalus and preventing ventriculitis simultaneously, or treating ventriculitis more effectively in case of infection which had already broken out. CLD has many advantages over EVD, although, it can complicate disastrous downward brain herniation in patients with elevated intracranial pressure. The authors performed CLD with EVD maintained and then tested the possibility of the brain herniation with quite simple method. If the CLD was proven as safe through the test, EVD could be replaced with it without terrible herniation. Material and Method : Between September 1998 and April 1999, 10 patients underwent CLD in replacement of EVD. Among them, 5 were patients with aneurysmal subarachnoid hemorrhage, 2 were patients with thalamic hematoma and intraventricular hemorhage and 3 were patients with traumatic intracranial hemorrhage. Results : In eight of them the replacements were successfully done and one of them died on account of medical illness. In two of them the replacement could not be performed because of the risk of herniation and all expired owing to ventriculitis. Two patients required permanent shunt operation. Conclusion : This article provides a valuable alternative method of treatment for persistent symptomatic hydrocephalus which can not be managed with shunt operation immediately.

• Clinical and Experimental Pediatrics
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• v.46 no.11
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• pp.1112-1117
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• 2003

Purpose : Recent advances in the methods of treating cancer in young patients have led to both an increased frequency of CNS complications as well as prolonged life expectancy. We intend to analyze the clinical aspects and laboratory findings of patients with CNS complications during and after treatment. Methods : We reviewed the medical records of 174 childhood cancer patients treated with chemotherapy admitted to the Dept. of Pediatrics, Keimyung University Dongsan Hospital, from January 1995 to November 2002. Among them, 15 cases with CNS complications were investigated in this study. Results : CNS abnormalities were found in 13 patients by CT or MRI during treatment such as leukoencephalopathy(n=7), mineralizing microangiopathy(n=4), brain infarction(n=3), intracranial hemorrhage(n=1), and hypoxic ischemic encephalopathy(n=1). It was found that two patients had two or more CNS abnormalities. Two patients who had no imaging abnormalities had convulsions, possibly after the addition of intrathecal methotrexate. The patients with intracranial hemorrhage and brain infarction had rapid and fatal clinical courses. The hypoxic ischemic encephalopathy following electrolyte imbalance completely recovered after correction of electrolyte. Conclusion : The CNS complications that occur during and after chemotherapy influence prognoses significantly, and remain neurologic sequelae. Therefore early diagnosis and prophylaxis for CNS complications and regular physical examination of patients who have recieved cancer therapy are strongly recommended.

• Journal of Chest Surgery
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• v.29 no.11
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• pp.1212-1217
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• 1996

From Dec. 1993 to May 1995, 9 male and 5 female patients ranging in age from 25 to 65 years, were operated on for ascending aorta and/or aortic arch diseases. Six patients had acute aortic dissection, type A(ruptured in 4 cases); four had ruptured ascending aortic aneurysm; three had annuloaortic ectasia(ruptured in 1 cases); one had aortic arch aneurysm. The diagnostic procedures were echo cardiography and dynamic CT scan in all patients having acute dissection or rupture. The aortic angiography was performed in two cases. Indications for operations were rupture in five cases, acute aortic dissection in five cases, severe congestive heart failure in two cases, progressive aortic insufficiency in one case and impending rupture in one case. The emergent repair was performed in ten cases(71%). The surgical treatment consisted of 6 Cabrol operations, a Cabrol operation combined with arch replacement, a modified Bentall operation, 4 replacement of ascending aorta, a replacement of aortic arch, and a replacement of ascending aorta and aortic arch. Complications were a hypoxic encephalopathy, two atrial fibrillations, a sternal deheiscence, and a mediastinitis. Two early mortality(14%) were due to intractable bleeding and multiple organ failure, and one late mortality(7%) was due to ventricular arrhythmia. In eleven survivors, follow-up period was from 2 months to 12 months and the course was uneventful.

• Neonatal Medicine
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• v.15 no.1
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• pp.61-66
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• 2008

Purpose : The purpose of this study was to describe the epidemiologic features of febrile illnesses in newborns and to predict the risk of serious infections in this population. Methods : A retrospective study was conducted on 123 full-term infants <30 days of age with an axillary temperature >38$^{\circ}C$ who were hospitalized between 2000 and 2006. Neonates with prenatal risk factors, congenital anomalies, antibiotic administration prior to admission to the hospital, or suspected hospital-acquired infections were excluded. We evaluated the symptoms, physical examination findings, laboratory data, and clinical course between the high- and low-risk groups for serious infections. Results : The high-risk group included 30 infants with the following diagnoses in order of frequency: aseptic meningitis, urinary tract infection, bacterial meningitis, infectious enteritis, sepsis concomitant with disseminated intravascular coagulopathy, bacteremia, pneumonia, cellulitis, and omphalitis. Leukocytosis and thrombocytopenia were statistically different between the two groups. Factors, such as moaning signs, seizures, body temperature, and pulse rate were statistically significant. Conclusion : Unlike previous studies, we included newborns with clinical bacterial infections and aseptic meningitis as the high-risk group. Leukocytosis, thrombocytopenia, moaning signs, seizures, and changes in vital signs were considered useful predictors for identifying febrile neonates at high-risk for serious infections in spite of a difference in the definition of serious infection.

• Journal of the Korean Child Neurology Society
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• v.26 no.4
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• pp.246-250
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• 2018

Purpose: Meningitis is an acute childhood infection caused by viral or bacterial infection. The purpose of the present study is to analyze the differences between enteroviral meningitis and non-enteroviral aseptic meningitis. Methods: From January 2013 to December 2016, we retrospectively reviewed the medical records of a total of 303 aseptic meningitis patients who visited Wonju Severance Christian Hospital. We examined demographics of all patients and analyzed serologic and cerebrospinal fluid tests, clinical symptoms, and outcomes. Results: Of a total of 303 patients, 197(65.0%) were male, and the most cases occurred from June to November (91.8%). The most common pathogen of meningitis was found to be enterovirus (65.0%). According to the etiology, the enteroviral meningitis group had significantly more headache and enteric symptoms (P=0.0003 and P=0.0013, respectively). Furthermore, the non-enteroviral meningitis group showed pleocytosis in the cerebrospinal fluid and a significantly higher rate of seizure at 1 to 4 years (P=0.0360 and P=0.0002, respectively). Conclusion: In this study, enteroviral meningitis was the most common and the prognosis was good. When compared between two groups, neurological symptoms were frequent in patients with non-enteroviral meningitis in groups 1 to 4 years.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.20 no.2
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• pp.37-43
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• 2020

Phenylketonuria is the most prevalent disorder caused by an inborn error in aminoacid metabolism. It results from mutations in the phenylalanine hydroxylase (PAH) gene. If untreated or late treated, results in profound and irreversible mental disability. Newborn screening test identify patients with phenylketouria. The early initiation of a phenylalanine restricted diet very soon prevents most of the neuropsychiatric complications. However, the diet therapy is difficult to maintain and compliance is poor, especially in adolescents and adulthood. Since 2015, American Medical College of Medical Genetics and Genomics (ACMG) recommended more strong restrictive diet therapy for target blood level of phenylalanine (<360 umol/L). For over four decades the only treatment was a very restrictive low phenylalanine diet. This changed in 2007 with the approval of cofactor therapy (Tetrahydrobiopterin, BH4) which is effective in up to 30% of patients. Data from controlled clinical trials with sapropterin dihydrochloride indicate a similar occurrence of all-cause adverse events with this treatment and placebo. Large neutral aminoacids (LNAA) competes with phenylalanine for transport across the blood-brain-barrier and have a beneficial effect on executive functioning. A new therapy has just been approved that can be effective in most patients with PAH deficiency regardless of their degree of enzyme deficiency or the severity of their phenotype. Phenylalanine ammonia lyase (PAL-PEG) was approved in the USA by FDA in May of 2018 for adult patients with uncontrolled blood phenylalanine concentrations on current treatment. Nucleic acid therapy (therapeutic mRNA or gene therapy) is likely to provide longer term solutions with few side effects.

• Clinical and Experimental Pediatrics
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• v.50 no.11
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• pp.1061-1066
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• 2007

Purpose : Early identification of developmental disabilities allows intervention at the earliest possible point to improve the developmental potential. The Ages and Stages Questionnaire (ASQ), a parent- completed questionnaire, can be used as a substitute for formal screening tests. The purpose of this study was to evaluate the validity of the Korean version of the ASQ (K-ASQ) as a screening tool for detecting developmental delay of young Korean children in the setting of a busy pediatric outpatient clinic. Methods : Parents completed the K-ASQ in the waiting room of the pediatric outpatient clinic of St. Mary's Hospital, Catholic University Medical College. Out of 150 completed the ASQ, 67 who were born term and had no previous diagnosis of developmental delay, congenital anomalies, or neurological abnormalities were enrolled. The cut-off values of less than 2 standard deviations (SD) below the mean for the ASQ were used to define a "fail", and children who failed in one or more domains tested were classified as "screen-positive". Diagnosis of developmental delay was made when the developmental indices fell below -1 SD of the Bayley Scales of Infant Development-II. Results : (1) The mean age of children was $16.4{\pm}7.4$ months. Ten children (14.9%) were small-for- gestational age infants. The mean birth weight and gestational age were $3.1{\pm}0.6kg$ and $38.8{\pm}1.4$ weeks. Nine children (13.4%) were twins and 33 (49.0%) were male. The mean maternal education in years was $13.6{\pm}2.4$, and 31.3% had full-time jobs. The time for completing the ASQ was $10.2{\pm}3.0$ minutes. (2) Seventeen children (25.4%) were classified as screen-positive, four of them were delayed in development. Among eight children diagnosed with developmental delay, four were screen-positive and the other four were screen-negative by the ASQ. (3) The test characteristics of the ASQ were as follows: sensitivity (50.0%); specificity (78.0%); positive predictive value (23.5%); negative predictive value (92.0%). Conclusion : The high negative predictive value of the K-ASQ supports its use as a screening tool for developmental delay in the setting of a pediatric outpatient clinic.

• Pediatric Infection and Vaccine
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• v.15 no.2
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• pp.121-128
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• 2008

Purpose : The seasonality of influenza virus and rotavirus are well recognized in winter and so viral surveillance and laboratory-based diagnostics are important to guide the timing of prophylaxis and other interventions. Yet the seasonality of these two viruses are changing in Korea. We evaluated the prevalence and clinical features of influenza virus and rotavirus. Methods : From September 2001 to August 2005, nasopharyngeal aspirates were cultured from the hospitalized patients with lower respiratory infections and the stools from hospitalized patients with gastroenteritis were tested for rotavirus. We retrospectively analysed the medical records. Results : During the study period, respiratory virus was isolated in 578 (18.5%) out of 3,121 patients. Influenza virus was isolated in 143 cases. The seasonal distribution of influenza infection was from December to June of the next year. The ratio of males to females was 1.3:1 and the median age was 17 months. The most common diagnosis of influenza infection was bronchiolitis. Fever and cough were present in 94.4% and 83.9% of the patients, respectively. During the same period, 3,850 patients were admitted for gastroenteritis and 1,047 (27%) patients were positive for rotavirus. Rotavirus was prevailed from December to June of the next year and it presented in year-round. The ratio of males to females was 1.1:1 and the median age was 16 months. Diarrhea and vomiting were the most common symptoms. Conclusion : The recent peak prevalence of influenza virus and rotavirus in Korea was in winter and the late spring, respectively. So we need to expand surveillance and carefully consider the correct period to vaccinate people.

• Radiation Oncology Journal
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• v.21 no.2
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• pp.107-111
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• 2003

Purpose: To establish the role of stereoactic radiosurgery using a linear accelerator for the treatment of patients with cavernous angloma. Materials and Methods: Between February 1995 and May 1997, 11 patients with cavernous angioma were treated with stereotactic radiosurgery using a linear accelerator. Diagnoses were based on the magnetic resonance imaging in 8 patients, and the histological in 3. The vascular lesions were located on the brainstem (5 cases), cerebellum (2 cases) thalamus (1 case) and cerebrum (3 cases). The clinical presentation at onset included previous intracerebral hemorrhages (9 cases) and seizures (2 cases). All patients were treated with a a linac-based radiosurgery. The median dose of radiation delivered was 16 Gy ranging from 14 to 24 Gy, which was typically proscribed to the 80$\%$ isodose surface (range 50 $\~$ 80$\%$), corresponding to the periphery of the lesion with a single isocenter. Ten patients were followed-up. Results: The median follow-up was 49 months ranging from 8 to 73 months, during which time two patients developed an intracerebral hemorrhage, 1 at 8 months, with the other at 64 months post radiosurgery. One patient developed neurological deficit after radiosurgery, and two developed an edema on the T2 weighted images of the MRI surrounding the radiosurgical target. Conclusion: The use of stereotactic radiosurgery in the treatment of a cavernous angioma may be effective in the prevention of rebleedlng, and can be safely delivered. However, a longer follow-up period will be required.

• Pediatric Infection and Vaccine
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• v.11 no.2
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• pp.158-169
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• 2004

Purpose : Although influenza is one of the most important cause of acute respiratory tract infections in children, virus isolation is not popular and there are only a few clinical studies on influenza and diagnostic methods. We evaluated the epidemiological and clinical features of influenza in children and rapid antigen detection test(QuickVue influenza test) on fist half of the year 2004 in Busan. Methods : From January 2004 to June 2004, throat swab and nasal secretion were obtained and cultured for the isolation of influenza virus and tested by rapid antigen detection test(QuickVue influenza test) in children with suspected influenza infections. The medical records of patients with influenza virus infection were reviewed retrospectively. Results : Influenza viruses were isolated in 79(17.2%) out of 621 patients examined. Influenza virus was isolated mainly from March to April 2004. The ratio of male and female with influenza virus infection was 1.2 : 1 with median age of 4 years 6month. The most common clinical diagnosis of influenza virus infection was bronchitis. There was no difference between influenza A and B infection in clinical diagnosis and symptoms. All patients recovered without severe complication. The sensitivity obtained for rapid antigen detection test (QuickVue influenza test) was 93.6% and the specificity was 80.2%, the positive predictive value 40.8%, the negative predictive value 98.8%. Conclusion : With rapid antigen detection test, it is possible early detection of influenza in children. reduction in use of antimicrobial agent and early use of antiviral agent.