Familial hemophagocytic lymphohistiocytosis (FHL) is a rare disease characterized by fever, hepatosplenomegaly, cytopenia and non-malignant lymphohistiocytic infiltration with hemophagocytosis in reticulendothelial organs. We experienced three cases of FHL in identical male twins and their younger brother who presented with fever and severe hepatosplenomegaly. Cytopenia, elevated serum transaminase and low serum albumin levels, hypertriglyceridemia were common laboratory findings of them. One of them showed markedly decreased phytohemagglutinin induced lymphocyte proliferation and reversed CD4/CD8 ratio (0.52) in flowcytometric lymphocyte subset analysis. Aspirate of bone marrow revealed typical features consistent with FHL in two of them. In spite of recent therapeutic approaches, none of them survived.
Bone Marrow ; Fever ; Humans ; Hypertriglyceridemia ; Lymphocyte Subsets ; Lymphocytes ; Lymphohistiocytosis, Hemophagocytic* ; Male ; Rare Diseases ; Serum Albumin ; Siblings

The fluoroquinolones are an important group of antibiotics widely used in the treatment of various infectious diseases in adults, as a result of an excellent spectrum of activity, good tissue penetration and convenient ways of administration. In recent decades, there has been extensive development, clinical investigation, licensure and use of fluoroquinolone antibiotics. However, the use of fluoroquinolones in children has been limited because of their potential to induce arthropathy in juvenile animals. Despite class label warnings against use in children, prescriptions for quinolone antibiotics to treat infections in children have become increasingly prevalent. The main use of fluoroquinolones in pediatrics should be, understandably, in serious life-threatening infections for which other antibiotics therapies are not effective or available. While most of the published studies failed to detect an increased rate of articular adverse effects in children treated with fluoroquinolones, an increase in the use of these compounds, particularly in community-acquired lower respiratory infections, could accelerate the emergence of multidrug-resistant (including fluoroquinolone) pneumococcal strains. This review will discus the main issues related to the use of fluoroquinolones in children, the major problems of resistance developing among these compounds, with special emphasis on the potential side effects and skilled use of these alternative potent drugs in pediatric infection.
Adult ; Animals ; Anti-Bacterial Agents ; Child ; Communicable Diseases ; Fluoroquinolones ; Humans ; Licensure ; Pediatrics ; Prescriptions ; Respiratory Tract Infections

To identify significant prognostic factors in patients undergoing simple hysterectomy in the presence of invasive cervical cancer, the records of 45 patients who had taken such a procedure between 1993 and 1997 were reviewed. Overall relapse-free survival and 5-year survival rates were 91.1 and 92.1%, respectively. Factors found to be significantly related to survival were the retrospectively determined stage(p=0.0000), the presence of residual disease(p=0.0001), and cell type(p=0.0000). By multivariate analysis, factor emerging as significantly detrimental to survival was the cell type. The presence of residual disease was a marginally significant factor(p=0.067). The expectations for survival of patients with residual tumor mass and/or with adenocarcinoma after simple hysterectomy appear to be markedly worse than those with others, so radical reoperation should be considered in those patients.
Adenocarcinoma ; Humans ; Hysterectomy* ; Multivariate Analysis ; Neoplasm, Residual ; Reoperation ; Retrospective Studies ; Survival Rate ; Uterine Cervical Neoplasms*

OBJECTIVES: Early diagnosis of intrauterine growth retardation is important to ensure optimal monitoring and delivery with the introduction of real-time and Doppler ultrasound systems, a noninvasive method of measuring human fetal blood flow has become available. The aim of this study is to compare blood flow velocity waveforms at the fetal descending aorta and umbilical artery in normal and in patients with pregnancy induced hypertension. METHODS: Using a combination of linear array real-time and pulsed Doppler ultrasound, blood flow velocity measurements were carried out at the fetal descending aorta and umbilical artery in 35 normal pregnancies and 18 cases of pregnancy induced hypertensive patients. RESULTS: The mean systolic/diastolic ratio of umbilical artery and aorta was significantly higher in PIH patients than in normal pregnancies(3.8 +/- 0.81 versus 2.97 +/- 0.52, p<0.05) and to predict perinatal morbidity, umbilical velocimetry is more sensitive than that of descending thoracic aorta. CONCLUSION: This study suggests that umbilical artery velocimetry could be used as a marker to predict adverse perinatal outcome.
Aorta ; Aorta, Thoracic* ; Blood Flow Velocity ; Early Diagnosis ; Female ; Fetal Blood ; Fetal Growth Retardation ; Humans ; Hypertension, Pregnancy-Induced ; Pregnancy ; Rheology* ; Ultrasonography ; Umbilical Arteries

Upper respiratory tract infection is one of the most common illnesses affecting children. On average, children experience around six to eight upper respiratory tract infections (URTIs) each year. Although these infections usually are mild and self limiting, they occasionally lead to complications that can be life threatening. Most URTIs can be placed within four main categories of infection: nasopharyngitis, pharyngitis, sinusitis and otitis media. Within each category of illness, there is a range of related conditions that may have similar or overlapping clinical presentations. A sound judgment is required to determine the most affected part of the respiratory mucosa. The clinical features, diagnosis and treatment of URTIs in children will be reviewed here.
Child ; Humans ; Judgment ; Nasopharyngitis ; Otitis Media ; Pharyngitis ; Respiratory Mucosa ; Respiratory Tract Infections ; Sinusitis

PURPOSE: Recently, the list of Epsterin-Barr Virus (EBV)-associated diseases has been growing. It is now known that there are two types (type 1 & 2) of EBV, but it has yet to be fully investigated as to whether type B EBV differs in any way from type A virus in terms of geographical distribution or disease association. We performed this study to find out the prevalence of the two types of EBV in EBV seropositive healthy children and adults by using one step Polymerase chain reaction(PCR). METHODS: Fifty-four viral capsid antigen (VCA) IgG positive healthy children (5 to 15 yrs of age) and 60 healthy adults (20 to 50 yrs of age) were involved in this study. DNA extracted from saliva samples of healthy study children and adults were subjected to amplification using the modified one step general PCR, which was developed by M. Kunimoto et al for detection of EBV types. And B95-82 cells (type 1) and Jijoye cells (type 2) were used as positive controls, and K-562 cells was used as a negative control. RESULTS: EBV was detected in mouth washing saliva of 8 (14.8%) of 54 healthy children. Five EBV detected cases contained type 1 and the remaining three cases contained type 2. EBV was detected in mouth washing saliva of 21 (35%) of 60 healthy adults. Twenty EBV detected cases contained type 1 and the remaining one was type 2. In total, EBV was detected in the saliva samples of 29 cases (25.4%), and 25 (86.2%) contained type 1. CONCLUSION: The results of our study indicate that type 1 is highly dominant in Korea, and EBV excretion is more frequent in EBV positive healthy adults compared to EBV positive healthy children in Korea.
Adult* ; Capsid ; Child* ; DNA ; Herpesvirus 4, Human* ; Humans ; Immunoglobulin G ; Korea ; Mouth ; Polymerase Chain Reaction ; Prevalence ; Saliva

OBJECTIVE: Although the use of cytology in screening has reduced morbidity and mortality from invasive cervical cancer, there has been limitation because of the low sensitivity and high false negative rate in the detection of preclinical or clinical disease of the cervix. This study was performed to explore the usefulness of Hybrid Capture System HPV DNA test as a diagnostic method of the cervical cancer. METHODS: Cervical cytology and Hybrid Capture System 1 (HCS1) HPV DNA test were performed in 1,023 women who visited Gynecologic Oncology Clinic in Guro Hospital, Korea University Medical Center. HPV DNA were retested by HCS1, HCS2 and Polymerase Chain Reaction(PCR), in randomly selected 200 women to compare these DNA tests. RESULTS: While the sensitivity and specificity of cervical cytology in detection of cervical lesion were 71.2% and 89.5%, those of HCS1 HPV DNA test were 63.0% and 86.9% respectively. There was significant difference in detection of cervical precancerous lesions between HCS1 and HCS2 (P<0.05), but not between HCS2 and PCR (P=0.14). And the sensitivity and specificity were improved with combining cytology and HCS1 HPV DNA test(82.2%, 86.9%~). CONCLUSION: HCS HPV DNA test may be considered as a useful adjunctive test with cervical cytology in cervical cancer screening.
Academic Medical Centers ; Cervix Uteri ; DNA ; Female ; Human Papillomavirus DNA Tests* ; Humans ; Korea ; Mass Screening* ; Mortality ; Polymerase Chain Reaction ; Sensitivity and Specificity ; Uterine Cervical Neoplasms*

Hypokalemic periodic paralysis (HOPP) is a rare disease characterized by reversible attacks of muscle weakness accompanied by episodic hypokalemia. Recent molecular work has revealed that the majority of familial HOPP is due to mutations in a skeletal muscle voltage-dependent calcium-channel: the dihydropyridine receptor. We report a 13-yr old boy with HOPP from a family in which 6 members are affected in three generations. Genetic examination identified a nucleotide 3705 C to G mutation in exon 30 of the calcium channel gene, CACNA1S. This mutation predicts a codon change from arginine to glycine at the amino acid position #1239 (R1239G). Among the three known mutations of the CACNA1S gene, the R1239G mutation was rarely reported. This boy and the other family members who did not respond to acetazolamide, showed a marked improvement of the paralytic symptoms after spironolactone treatment.
Acetazolamide/pharmacology ; Adolescent ; Arginine/chemistry ; Calcium Channels/chemistry/*genetics ; Codon ; Exons ; Family Health ; Female ; Glycine/chemistry ; Humans ; Hypokalemia/metabolism ; Hypokalemic Periodic Paralysis/*diagnosis/*genetics ; Korea ; Male ; Muscle, Skeletal/metabolism ; Mutation ; Pedigree ; Protein Structure, Tertiary ; Sequence Analysis, DNA ; Spironolactone/pharmacology

The immunogenicity of a single dose of Salmonella typhi(S.typhi) Vi capsular polysaccharide(CPS) vaccine was evaluated before, and at 1, 3, 12, and 36 months after vaccination. Eighty-five adults(20-28 years of age) and sixty-four children(8-16 years of age) received a single dose of 25 micrograms Vi CPS vaccine intramuscularly, and antibody titers to Vi CPS were measured by passive hemagglutination. Of 149 vaccinees, 138(92.6%) showed seroconversion at 1 month after vaccination, and then 138 out of 141(97.9%) did at 3 months. Of 137 vaccinees, 116(84.7%) maintained a persistent rise in Vi antibody titer 12 months after vaccination, and 55 out of 100(55.0%) had a 4-fold or greater rise at 36 months. No significant adverse reactions were observed. Booster injection may be needed 3-5 years after vaccination.
Adolescent ; Adult ; Antibodies, Bacterial/blood ; Antigens, Bacterial/immunology ; Child ; Evaluation Studies ; Human ; Polysaccharides, Bacterial/*immunology ; Salmonella typhi/*immunology ; Typhoid Fever/prevention & control ; Typhoid-Paratyphoid Vaccines/adverse effects/immunology/*therapeutic use

PURPOSE: We investigated incidence of feeding intolerance and time when normal enteral feeding can be established in full term neonates with hypoxic-ischemic encephalopathy (HIE). METHODS: We reviewed medical records of 61 full term infants with HIE who were admitted to St. Paul's Hospital from Jan. 1996 to Dec. 2001. The incidence of feeding intolerance, day of first feeding, and day of full enteral feeding were studied in respective to Sarnat stages. RESULTS: Among the full term with HIE, 32 were classified into stage I, 20 into stage II, and 9 into stage III. The incidence of feeding intolerance was 6%, 30%, and 89% for stages I, II and III, respectively. There was only one case of necrotizing enterocolitis among infants of stage III HIE. Feeding first began on 0.13+/-0.01 postnatal day (PND) in normal infants compared to 0.15+/-0.03 PND in infants of stage I, 3.24+/-1.82 PND in stage II and 5.58+/-2.50 PND in stage III. The incidence of feeding intolerance, day of first feeding, and day of normal enteral feeding achieved in infants with stage I were not different from those of normal infants but significantly higher and delayed in infants with more severe degrees of encephalopathy. CONCLUSION: The first feeding should vary according to severity of encephalopathy so as to lower the incidence of feeding intolerance and the risk of necrotizing enterocolitis. We suggest that infants of stageIencephalopathy be first fed as same as normal infants, but precaution is in order when deciding an appropriate time to start feeding in infants of stage II, III encephalopathy.
Enteral Nutrition ; Enterocolitis, Necrotizing ; Humans ; Hypoxia-Ischemia, Brain* ; Incidence* ; Infant ; Infant, Newborn* ; Medical Records