Murine typhus is a flea-borne infectious disease caused by Rickettsia typhi, of which myocarditis is a rare complication in the acute disseminating phase. A 62-year-old female presented with a fever and was diagnosed with murine typhus. She was treated with doxycycline and discharged after complete resolution of the fever. However, recurrent presyncope and exertional dyspnea developed 6-8 weeks later. Complete atrioventricular (AV) block with a wide QRS escape rhythm and a left bundle branch block configuration was documented. Subacute myocarditis was diagnosed based on persistent cardiac troponin-I elevation and typical cardiac magnetic resonance imaging findings. A permanent pacemaker was implanted for symptomatic complete AV block. Few reports of myocarditis in murine typhus have been published. We report a case of murine typhus myocarditis complicated by complete AV block in the late convalescence phase.
Atrioventricular Block ; Bundle-Branch Block ; Communicable Diseases ; Convalescence ; Doxycycline ; Dyspnea ; Female ; Fever ; Humans ; Magnetic Resonance Imaging ; Myocarditis ; Rickettsia typhi ; Syncope ; Troponin I ; Typhus, Endemic Flea-Borne ; United Nations

BACKGROUND AND OBJECTIVES: With the wide use of cochlear implants, there have been many published studies which report that cochlear implants have a suppressive effect on tinnitus in profoundly deaf patients. The aim of this study was to understand the clinical and audiologic characteristics of the tinnitus in patients with cochlear implantation and to observe changes of their tinnitus after cochlear implantation. SUBJECTS AND METHOD: A total 23 patients who had undergone cochlear implants in our hospital from August 2003 to December 2006, were included in this study. The patients were divided into tinnitus (n=11) and non-tinnitus groups (n=12) according to the presence of tinnitus at the time of surgery. We compared clinical and audiologic features between the two groups. We also studied changes in tinnitus after surgery in the tinnitus group using validated self-report measures [tinnitus handicap inventory (THI), tinnitus handicap score (THS), and visual analogue scale]. RESULTS: There were significant differences between the two groups with respect to the age of cochlear implant recipients, whether or not the subjects had impaired hearing or were wearing hearing aids. Tinnitus was rarely observed in patients under 20 years of age with cochlear implantation (11.1%) in contrast to the higher rates in the patients of 20 years old and over (71.4%). Marked reduction of THI, THS and visual analogue scale of tinnitus were observed in all patients. CONCLUSION: For the adult patients who had undergone cochlear implant surgery and had tinnitus preoperatively, cochlear implants had shown suppressive effect on their tinnitus. Considering the significant prevalence of tinnitus in profoundly deaf patients, tinnitus of these patients should not be overlooked and further studies should be made to define relationship between cochlear implant and tinnitus.
Adult ; Cochlear Implantation ; Cochlear Implants ; Hearing ; Hearing Aids ; Humans ; Prevalence ; Tinnitus

PURPOSE: Mitochondrial disorders are a clinical entity characterized by diverse symptoms and signs of involvement of various systems. Furthermore, the disorders are known to show ophthalmologic manifestations as well as neurological findings. Visually evoked potential is a sensitive measure to check the integrity of the visual pathway. In this study, we have investigated the value of visually evoked potential in mitochondrial disorders with respiratory chain defects. METHODS: Nineteen patients diagnosed with mitochondrial respiratory chain complex I defect as confirmed by spectrophotometric enzyme assay in muscle samples were enrolled for this study. The patients underwent a visually evoked potential study. We classified the results into four groups and compared these with clinical ophthalmologic findings. RESULTS: Among the 19 patients, 14 showed abnormal visually evoked potential findings. Seven patients showed abnormal clinical ophthalmologic findings. All patients with abnormal ophthalmologic findings showed abnormal visually evoked potential findings. Among the 12 patients with normal ophthalmologic findings, seven showed abnormal results in visually evoked potential. CONCLUSION: Visually evoked potential study could be used as an effective screening tool for mitochondrial disorders to detect ophthalmologic and neurological abnormalities.
Child ; Electron Transport ; Enzyme Assays ; Evoked Potentials ; Eye ; Humans ; Mass Screening ; Mitochondria ; Mitochondrial Diseases ; Muscles ; Visual Pathways

OBJECTIVETo evaluate the safety of a group A + C meningococcal polysaccharide vaccine as part of a phase IV clinical trial.

METHODSThe study area was divided into 108 clusters according to the principle of cluster randomization, stratified and paired sampling methods. 54 out of 108 clusters served as observation groups were administered A + C vaccine, while the rest 54 groups were administered Vi polysaccharide vaccine. An adverse event surveillance system was established to monitor the adverse events following the vaccination campaign. Identical form and methods were used for data collection to investigate the adverse events following the vaccination of both A+ C vaccine and Vi vaccine.

RESULTS34,543 people were vaccinated, including 18,167 of whom received A + C vaccine, while the other 16,376 received Vi vaccine. The rates of immediate injection reaction and unsolicited non-serious adverse events from A + C vaccine group were 0.44% and 0.38% while of Vi vaccine group were 0.79% and 0.73% respectively. At the solicited adverse event survey on 3-day-post-vaccination, 1239 vaccinees were followed-up including 771 received A + C vaccine and 468 received Vi vaccine. The local injection reaction rate of A + C vaccine group on the 1st day was significantly higher (X2 = 13.98, P = 0.0002) than that of Vi vaccine group. Neither the local injection reaction rate nor the system reaction rate between both groups was significantly different on 2nd and 3rd day, post vaccination. It was not statistically different when comparing fever onset rate between those who received vaccine and those who did not, in each vaccine group. There were no serious adverse events observed.

CONCLUSIONResults showed that the side effects of A + C vaccine and the Vi vaccine were mild and safe for vaccination campaigns targeting on populations at different age.

Adolescent ; Adult ; Age Distribution ; Child ; Child, Preschool ; Female ; Humans ; Male ; Meningococcal Vaccines ; adverse effects ; immunology ; Middle Aged ; Polysaccharides, Bacterial ; immunology ; Sex Distribution ; Young Adult

BACKGROUND AND OBJECTIVES: Various surgical approach has been used for the removal of pituitary tumor. Recently, endoscopic transnasal transsphenoidal approach has been technically upgraded and the morbidity associated with surgical treatment of the pituitary tumor has been decreased. The objectives of our study was to describe surgical techniques and materials used in sellar repair after endoscopic transnasal transsphenoidal approach. SUBJECTS AND METHOD: We retrospectively reviewed the medical records of 18 patients who had received endoscopic transnasal transsphenoidal pituitary surgery between November 2002 and January 2004. We evaluated effectiveness of this technique by analyzing surgical techniques, symptom improvement and complications after surgery. RESULTS: In most of the case, tumor was found to be macroadenoma and 8 of the cases had suprasellar extension. Tumor was totally removed in 13 cases and partially removed in 5 cases. In all cases we used endoscopic unilateral transnasal transsphenoidal approach with anterior sphenoidotomy. CONCLUSION: We may consider that this approach is more safe and effective and a better treatment method for pituitary tumor surgery than the transcranial approach.
Endoscopy ; Humans ; Hypophysectomy* ; Medical Records ; Pituitary Neoplasms* ; Retrospective Studies

Intraoperative pulmonary thromboembolism (PTE) is rare, but is nevertheless one of the important causes of morbidity and mortality in patient undergoing surgery. However, The detection of PTE is difficult because it's clinical symptoms and signs are nonspecific during surgery, and specialized diagnostic tools are not readily available in the operating room. We report a case of PTE due to cancer emboli with thrombi encountered during a nephrectomy in patient with renal cell cancer, and demonstrate that one-lung ventilation can aid in the diagnosis of massive PTE when the evaluation is suspected.
Carcinoma, Renal Cell ; Diagnosis ; Humans ; Mortality ; Nephrectomy ; One-Lung Ventilation* ; Operating Rooms ; Pulmonary Embolism*

BACKGROUND AND OBJECTIVES: For patients with sudden hearing loss or retina arterial spasm, stellate ganglion block (SGB) has been used as a treatment method to increase the blood flow to the interested area. The aim of this study is to prospectively investigate the efficacy of SGB in patients with olfactory dysfunction following upper respiratory tract infection (URI). MATERIALS AND METHOD: Fifty one patients with anosmia or hyposmia following URI were included. The average duration of olfactory dysfunction was 3.5+/-8.4 years. Thirty eight patients were treated with SGB and compared with the untreated 13 patients. Buthanol threshold test and odor identification test with 16 natural fragrances were used to determine anosmia/hyposmia in these patients and to quantify the improvement of olfactory perception after SGB treatment or follow-up without treatment. RESULTS: Olfactory perception was improved significantly in the treated patients as shown by the buthanol threshold test (p<0.001) and by odor identification test (p<0.001). Subjective olfactory perception was improved in 27 of the 38 treated patients (p<0.001). No complications were observed after SGB and no one reported any worsening in olfactory perception during or after treatment. On the other hand, none of the untreated patients showed any improvement in neither of the tests or the subjective assessment. CONCLUSION: These results suggest that SGB may be a new treatment modality for olfactory dysfunction following URI.
Follow-Up Studies ; Hand ; Hearing Loss, Sudden ; Humans ; Odors ; Olfaction Disorders ; Olfactory Perception ; Prospective Studies ; Respiratory System* ; Respiratory Tract Infections* ; Retina ; Smell ; Spasm ; Stellate Ganglion*

BACKGROUND: Congenital adrenal hyperplasia (CAH) results from an inherited defect in enzymatic steps required to synthesize cortisol from cholesterol. 21-hydroxylase deficiency accounts for 95% cases of CAH. It appears that the frequency and the type of the responsible mutations differ according to the ethnic background and the type of mutation can predict the clinical outcomes such as salt losing type (SL), simple virilizing type (SV) and non-classic type (NC). METHODS: We have analyzed CYP21 genes in 55 Korean cases (110 chromosomes) of CAH by Southern blotting, PCR-dot hybridization and PCR amplification-created restriction site method. The patients include 43 cases of SL and 12 of SV. None of the NC was found. RESULTS: We found the mutations in 94% (103/110) of the examined chromosomes. A total of 10 types of mutations were discovered. The mutations include aberrant splicing of intron 2 (i2, 35%), CYP21 gene deletion (32%) and I172N (11%) in order. When the relationship between the clinical types and genotypes were correlated, most of the SL patients have either i2 (42%) or CYP21 gene deletion (41%), while SV patients have I172N (33%) or P30L (21%). The parents' mutation was investigated in 20 cases. In 4 families, one of the parents was not the obligatory heterozygote carrier i.e. did not have a mutation. The results suggest the high incidence of de novo mutation. CONCLUSION: We have identified the frequency of mutations of the CYP21 in Korean AGS patients. Our results shows that the clinical type of AGS can be predicted from the genotypes of CYP21. Also the high incidence of de novo mutation of CYP21 confirmed the genetic instability of major histocompatibility III region where the CYP21 is located.
Adrenal Hyperplasia, Congenital ; Adrenogenital Syndrome* ; Blotting, Southern ; Cholesterol ; Gene Deletion ; Genotype* ; Heterozygote ; Histocompatibility ; Humans ; Hydrocortisone ; Incidence ; Introns ; Parents ; Phenotype* ; Polymerase Chain Reaction ; Steroid 21-Hydroxylase*

No abstract available.
Wilms Tumor*

BMP-4 signaling is mediated through Smad proteins which may translocate to the nucleus to activate transcription. Little is known about how BMP-4 signaling regulates the transcription of its target genes, e.g., Xvent genes. Therefore, we isolated the genomic clone of a BMP-4 responsive homeobox gene, Xbr-1a/Xvent-2. This clone contains a promoter and three exons for the entire coding region. Using the primer extension, we identified the transcription initiation site corresponding to position -64 bp upstream to the ATG codon of the Xvent-2 gene. The promoter was linked to the luciferase reporter gene, and promoter activity determined by luciferase assay. The temporal promoter activity peaked between embryonic stages 13~17, in agreement with its temporal mRNA expression in the whole embryo. Through the serial deletion mutation, the upstream -235 bp of the promoter retains the full transcriptional activity, and is regulated by BMP-4 signaling. The present results suggest that the BMP-4 responsive element is located on the upstream 235 bp of the promoter.
Clinical Coding ; Clone Cells ; Codon ; Embryonic Development* ; Embryonic Structures ; Exons ; Female ; Genes, Homeobox ; Genes, Reporter ; Luciferases ; Pregnancy ; RNA, Messenger ; Sequence Deletion ; Smad Proteins ; Transcription Initiation Site ; Xenopus laevis ; Xenopus*