No abstract available.
Epithelium* ; Humans ; Severe Combined Immunodeficiency*

No abstract available.

Nitroblue tetrazolium dye reduction test is now widely used for early detection of bacterial infection. Practically nitroblue tetrazolium dye reduction test (N.B.T. test) gives certain clue to rule out any possibility of aseptic inflammation. Authors performed N.B.T. test for 80 orthopaedic diseases and 40 controls, and the results were as followings: 1. Forty-one of 60 subjects with bacterial infection had more than 10% formazan positive neutrophils, and 18 of 20 subjects without bacterial infection showed less than 10%. formazan positive neutrophils, The groups of the elevated N.B.T. responses were mainly the subjects who had osteomyelitis, soft tissue inflammation, pyogenic arthritis, and bone and joint tuberculosis. 2. The mean proportion of N.B.T. positive neutrophils was 3.8±2.78% in 40 controls, 4.7±4.23% in 20 nonifectious group 12.7±10.3% in 12 chronic osteomyelitis, 12.5±10.7% in 15 treated bone and joint tuberculosis, 20.9±15.65% in 15 untreated bone and joint tuberculosis and 20±10.70% in 11 pyogenic arthritis and 7 other soft inflammatory group. 3. In noninfectious group, 7 of 8 subjects with elevated ESR and 3 of 4 subjects with elevated body temperature revealed N.B.T. responses less than 10%. 4. In 23 subjects with the elevated N.B.T. responses as well as leukecytosis, 22 subjects were the groups of bacterial infection and rest 1 was noninfectious in nature. 5. Therefore, the N.B.T. test is found to be useful for the purposes of the early diagnostic aid of the bacterial infection and differential diagnosis between bacterial infections and other diseases.
Arthritis ; Bacterial Infections ; Body Temperature ; Diagnosis, Differential ; Inflammation ; Neutrophils ; Nitroblue Tetrazolium ; Osteomyelitis ; Tuberculosis, Osteoarticular

BACKGROUND: Healthcare-associated infections (HAIs) are among the most important threats to patient safety. When hospitalized children face these threats, there is morbidity, mortality, prolonged hospitalization, and increased healthcare costs. Research on local healthcare epidemiology is necessary to enhance collective knowledge and evidence formanaging this problem. METHODS: We performed a retrospective analysis of databases of patients who were diagnosed with HAIs at Chung-Ang University Hospital (CAUH) from 2007 through 2011. Cases were selected from the microbiology registry databases. The data on prevalence of HAIs in various wards and its annual trends were compared to previously reported nationwide data. Moreover, we analyzed the patterns of antibiotic susceptibility results for HAI pathogens. RESULTS: A total of 181 HAIs were identified in 122 patients. The HAI rate among pediatric patients at CAUH was 2.4/1,000 person-hospital days. Urinary tract infections (UTIs) (53 episodes, 29.3%) were the most common, followed by pneumonia (33 episodes, 18.2%). Staphylococcus aureus was found to be the most common gram-positive organism, whereas Escherichia coli was the most common gram-negative organism. Methicillin-resistant S. aureus (MRSA) comprised 84% of the S. aureus infections. Imipenem resistance was detected in 58.8% and 55.0% of Acinetobacter baumannii and Pseudomonas aeruginosa isolates, respectively. CONCLUSION: Between 2007 and 2011, UTIs were the most common type of HAIs, and MRSA was the most common pediatric HAI pathogen, both in the general ward and intensive care unit at the CAUH. Further research on the epidemiology and pathogenesis of HAIs is necessary and prevention measures should be implemented to prevent HAIs in children.
Acinetobacter baumannii ; Child ; Child, Hospitalized ; Delivery of Health Care ; Escherichia coli ; Health Care Costs ; Hospitalization ; Humans ; Imipenem ; Intensive Care Units ; Methicillin Resistance ; Methicillin-Resistant Staphylococcus aureus ; Microbial Sensitivity Tests ; Patient Safety ; Patients' Rooms ; Pneumonia ; Prevalence ; Pseudomonas aeruginosa ; Retrospective Studies ; Staphylococcus aureus ; Urinary Tract Infections

C1q nephropathy is a rare glomerular disease, defined by characteristic mesangial C1q immune deposition seen in immunofluorescence microscopy with no serological evidence of systemic lupus erythematosus. C1q nephropathy can be diagnosed with a subsequent biopsy, as with IgA nephropathy. There are some cases with an initial diagnosis of hematuria and proteinuria with minimal disease changes, focal segmental glomerulonephritis, and mesangial proliferative glomerulonephritis, but lacking C1q nephropathy, in which C1q deposition on immunofluorescence subsequently develops. We report a case that was diagnosed as diffuse mesangial proliferative glomerulonephritis, but a subsequent biopsy showed C1q nephropathy, with C1q deposition in both immunohistochemistry and electron microscopy (EM). We treated the C1q nephropathy with methylprednisolone and confirmed the disappearance of C1q depositions by both immunohistochemistry and EM in a follow-up biopsy.
Biopsy ; Complement C1q ; Diagnosis ; Fluorescent Antibody Technique ; Follow-Up Studies ; Glomerulonephritis ; Glomerulonephritis, IGA ; Hematuria ; Immunohistochemistry ; Lupus Erythematosus, Systemic ; Methylprednisolone ; Microscopy, Electron ; Microscopy, Fluorescence ; Proteinuria

Percutaneous balloon valvuloplasty (PBV) was done in 7 adults of pulmonary valvular stenosis, which was diagnosed with catheterization and cine-angiography of rigtht heart, in Seoul National University Hospital from Jan. 1986 to Dec. 1986. The results were as follows : 1) Among the seven cases six were male and the range of age was from 19 to 40 years. Atrial septal defects associated in 2 cases were not significant clinically. 2) The peak systolic gradient from right ventricle to pulmonary artery before the procedure was below 50 mmhg in 1 cases, 50 to 100 mmHg in 1 case, and above 100 mmHg in 5 cases. 3) Before PBV the mean systolic pressure of right ventricle was 121+/-42 mmHg and the peak systolic gradient was 106+/-41 mmHg. After PBV those were 58+/-30 mmHg and 40+/-31 mmHg, revealing significant improvement. 4) Balloon catheter (Medi-tech, 9F) was used in all cases. single balloon of 20 mm outer diameter was applied in 2 cases and double balloons of various sizes were applied in 5 cases in which single balloon showed inadequate dilatation. It is suggested that percutaneous balloon valvuloplasty is safe and effective alternative to surgical valvotomy of pulmonary valvular stenosis in adults.
Adult* ; Balloon Valvuloplasty* ; Blood Pressure ; Catheterization ; Catheters ; Constriction, Pathologic* ; Dilatation ; Heart ; Heart Septal Defects, Atrial ; Heart Ventricles ; Humans ; Male ; Pulmonary Artery ; Seoul

Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS) is a slowing progressive ataxic disorder characterized by bilateral vestibulopathy, cerebellar ataxia and somatosensory impairment. Autonomic dysfunction is recently considered as a core feature in CANVAS in addition to these symptoms. In most cases, patients with CANVAS show cerebellar atrophy in brain imaging, but some cases show minimal or no atrophy of cerebellum. Brain (18F)-fluoro-2-deoxy-D-glucose positron emission tomography (¹⁸F-FDG PET) study can be a complimentary tool to diagnosis CANVAS in cases of no structural abnormality such as cerebellar atrophy. Hereby, we present a case of CANVAS with minimal atrophy of cerebellum but showing a prominent hypometabolism in cerebellum, thalamus and posterior cingulate cortex in ¹⁸F-FDG PET.
Atrophy ; Brain ; Cerebellar Ataxia ; Cerebellum ; Diagnosis ; Gyrus Cinguli ; Humans ; Neuroimaging ; Positron-Emission Tomography ; Primary Dysautonomias ; Thalamus ; Vestibular Neuronitis

BACKGROUND: Familial hypercholesterolemia(FH) is an autosomal dominant metabolic disorder caused by the mutation in low density lipoprotein receptor(LDLR) gene. However, direct genetic diagnosis of LDLR gene mutation is not easily available because more than 300 mutations have been described in LDLR gene of FH patients. Therefore indirect genetic diagnosis using the genetic markers can be used to follow the inheritance of defective gene in FH families. The purpose of this study was to evaluate the usefulness of indirect genetic markers for detecting identical-by-descent LDLR gene abnormalities in FH families. METHODS: We examined the allele frequency, heterozygosity, polymorphism information content(PIC) of each genetic markers(D19S394, Taq I, Hinc II, Ava II, ATn, D19S221) in 94 unrelated healthy subjects. The genetic polymorphic haplotypes in 3 FH families were also determined. RESULTS: The heterozygosity and PIC values of RFLP's(Taq I, Hinc II, Ava II) were 0.51/0.344, 0.25/0.223, 0.28/0.233 and microsatellite markers(D19S394, ATn, D19S221) were 0.64/0.558, 0.56/0.455, 0.60/0.475. Hinc II and Ava II were significantly linked(|D|=0.72, p< 0.05). The cumulative PIC values of Taq I+Hinc II, Taq I+Hinc II+ATn, D19S394+ATn were 0.520, 0.814, 0.813, respectively. When applied in the FH pedigree, the genetic diagnosis using only one marker was not available in most cases. However, combination of two or more genetic markers could successfully discriminate the affected and unaffected members in FH families. Among the several combinations of the genetic markers, the combination of D19S394 and ATn was supposed to be the most effective and informative. Because one case of recombination was suspected in D19S221 allele, it was thought to be carefully used for genetic diagnosis of FH. CONCLUSION: We concluded that indirect genetic diagnosis using intragenic or extragenic genetic markers was useful for detecting identical-by-descent LDLR gene abnormalities in FH families and the most effective and informative combination of genetic marker seemed to be D19S394 and ATn.
Alleles ; Diagnosis* ; Gene Frequency ; Genetic Markers* ; Haplotypes ; Humans ; Hyperlipoproteinemia Type II* ; Lipoproteins* ; Microsatellite Repeats ; Pedigree ; Receptors, Lipoprotein* ; Recombination, Genetic ; Wills

PURPOSE: To report the decreasing incidence of HBV(Hepatitis B virus)-associated membranous nephropathy in children after HBV vaccination and to elucidate the clinical course and treatment strategies of IMN (Idiopathic membranous nephropathy). METHODS: We retrospectively reviewed the clinico-pathological findings of HBV-MN and IMN patients who underwent a renal biopsy from 1986 to 2005. We compared the HBV-MN and the IMN groups and the remission and the non-remission groups of patients with IMN. RESULTS: Among 24 cases of MN patients, HBV-MN comprised 6 cases(25%) and IMN 18 cases(75%). Clinical masnifestations were nephrotic syndrome(3 cases, 50%), nephritic syndrome(1 case, 16.7%), asymptomatic(2 cases, 33.4%) in the HBV-MN group, asymptomatic(10 cases, 55.5%), nephrotic syndrome(5 cases, 27.8%), and gross hematuria(3 cases, 16.7%) in the IMN group. From 1996 to 2000, there were 2 cases(28%) of HBV-MN and 5 cases(72%) of IMN. After 2001, all 10 cases were IMN. In the HBV-MN group, 4 cases(66.7%) received interferon and 1 case received methylprednisolone pulse therapy. In the IMN group, 16 cases (88.9%) received methylprednisolone, 8 cases(44.4%) were in complete remission, 2 cases (11.1%) were in partial remission, 2 cases(11.1%) were in chronic renal failure, and 5 cases (27.8%) were lost to follow-up with sustained proteinuria, 1 case(5.6%) continued to have frequent relapse of nephrotic syndrome without renal insufficiency. In the comparison between remission and non-remission groups, nephrotic range proteinuria and hypertension were more significantly common in the non-remission group(P<0.05). CONCLUSION: With HBV vaccination, HBV-MN has decreased markedly. IMN is a rare glomerular disease in children. Because the prognosis for patients with nephrotic range proteinuria is poor, this group needs more aggressive treatment.
Biopsy ; Child* ; Glomerulonephritis, Membranous* ; Humans ; Hypertension ; Incidence ; Interferons ; Kidney Failure, Chronic ; Lost to Follow-Up ; Methylprednisolone ; Nephrotic Syndrome ; Prognosis ; Proteinuria ; Recurrence ; Renal Insufficiency ; Retrospective Studies ; Vaccination

Corrected transposition of the great arteries is usually characterized by inverted ventricles and transposition of the great arteries. This combination without cardiac anormalies results in normal arterial oxigen content, hence the term corrected. Unfortunately, the condition rarely exists without other major cardiac anormalies. The authors report a case of situs inversus that was associated with corrected TGA, VSD, PS and ASD. The girl was admitted for evaluation of systolic ejection murmur on the both lower sternal border. Doppler echocardiography and cardiac angiography showed the diagnosis and so valvulotomy and closure operation of VSD and ASD was done.
Angiography ; Arteries ; Diagnosis ; Echocardiography, Doppler ; Female ; Humans ; Situs Inversus ; Systolic Murmurs