The pupose of this study is to verify the role of urinary sodium in transient renal acidification defect which frequently combine acute infantile gastroenteritis. We studied on twenty-five infants, 2month to 36 month of age, during the 4 month period, from August, 1991 to December, 1991. The patients had acidosis for a mean of 3 days and sixty urine samples were collected during this period. The mean pH of 23 rine samples with sodium concentration<10 mmol/L was significantly higher than pH of 37 samples with sodium concentration<10 mmol/L, We separately analyzed 15 urine samples collected during metabolic acidosis after completion. of rehydration. The result was that a urinary acidification defent was observed in the urine samples with low sodium concentration but not in the sodium rich samples. We concluded that impaired urinary acidification defect is frequently during metabolic acidosis in infants with acute gastroenteritis and results from a sodium deficit rather than from transient distal renal tublur acidosis.
Acidosis ; Fluid Therapy ; Gastroenteritis* ; Humans ; Hydrogen-Ion Concentration ; Infant ; Sodium*

No abstract available.
Humans ; Infant, Newborn* ; Rhabdomyoma*

Arteriovenous fistulae(AVF) are well documented complications of percutaneous renal biopsy. Between August 1995 and April 1997, we prospectively studied 280 consecutive ultransound-guided percutaneous renal biopsies to evaluate the incidence of post-biopsy AVF and its natural course. All biopsies patients were monitored through a routine follow-up echo-color popple sonography at post-biopsy 1, 7, 14 and 30 days. The 28 patients(10%) out of 280 consecutive percutaneous renal biopses were diagnosed to have AVF. There was no differences in the incidence of AVF between native and transplanted kidney biopsy. Most AVF were small(<2cm) and caused minimal or no symptoms. AVF was accompanied with large hematoma(thickness above 2cm) was observed in 9 cases(53%) of 17 native kidneys and none in 10 transplanted kidneys. This difference was statistically significant(P=0.008). At follow-up, AVF resolved spontaneously in 24 cases(87%). In two patients(7.1%) required superselective arterial embolization, one develoved spontaneous rupture of AVF, and one had persistence of AVF over 12 months. We concluded that color Doppler sonography provides a good, non-invasive and safe method for diagnosis and follow up of post-biopsy AVF.
Arteriovenous Fistula ; Biopsy ; Diagnosis ; Fistula* ; Follow-Up Studies ; Humans ; Incidence* ; Kidney ; Prospective Studies ; Rupture, Spontaneous

PURPOSE: Early assessment of injury severity is important in management of major trauma patients. In general, hypotensive major trauma patients show more severe outcomes from injuries compared with normotensive major trauma patients. In this study, we analyzed the clinical features of severe trauma patients with initial hypotension and attempted to determine the prognostic factors of mortality in these patients. METHODS: A retrospective study was conducted within our hospital. Review of trauma registry data identified 679 major trauma patients (Injury severity score, ISS>15). From January 2011 to December 2013, all major trauma patients with initial systolic blood pressure lower than 90 mmHg were included (N=77). The patients were divided into two groups - those who survived and those who expired - and the differences in initial and final values were compared between the two groups. RESULTS: Out of a total of 77 patients, 55 patients survived and 22 patients died. The data showed almost no difference in heart rate between the two groups. The expired group showed low Glasgow Coma Scale (GCS) score, systolic blood pressure, revised trauma score, initial pH, and follow-up pH, as well as higher age, ISS, initial lactate, prothrombin time (PT), international normalized ration (INR), and follow-up lactate, compared with the survived group. In multivariate logistic analysis, age (p=0.034, OR 1.071), GCS (p=0.006, OR 0.61), initial base excess (p=0.042, OR 0.57), and follow-up base excess (p=0.041, OR 0.799) were independently associated with mortality. CONCLUSION: The patient's age, initial GCS, initial base excess and follow-up values of base excess were good prognostic factors for mortality in the expired major trauma patients with initial hypotension.
Blood Pressure ; Emergency Service, Hospital* ; Follow-Up Studies ; Glasgow Coma Scale ; Heart Rate ; Humans ; Hydrogen-Ion Concentration ; Hypotension* ; Lactic Acid ; Mortality* ; Multiple Trauma ; Prothrombin Time ; Retrospective Studies

No Abstract Available.
Female ; Hormone Replacement Therapy* ; Humans ; Life Style* ; Osteoporosis*

Huntington's disease(HD) is an autosomal dominantly inherited neurodegenerative disease, which is characterized by choreic movement and progressive dementia. HD is a disease caused by CAG repeat expansion of huntintin gene and definitely diagnosed or is excluded by molecular genetic analysis. Juvenile HD, of which onset is in children or young adult, is the most severely disabled type and shows several distinct clinical and genetic features in contrast to usual late-onset type. We report a 10 year-old girl who presented with involuntary movement, seizure and moderate dysarthria confirmed by molecular genetic analysis.
Child ; Chorea ; Dementia ; Dysarthria ; Dyskinesias ; Female ; Humans ; Molecular Biology ; Neurodegenerative Diseases ; Seizures ; Young Adult

Distraction osteogenesis (DO) is a surgical method of bone formation that involves an osteotomy and sequential stretching of the healing callus by gradual movement and subsequent remodeling. DO is used to correct facial asymmetry, such as in patients with hemifacial microsomia, maxillary or mandibular retrusion, cleft lip and palate, alveolar defects, and craniofacial deficiency. It is accomplished with the aid of a distraction device, which is secured with screws placed directly into bone, for a predetermined length of time. Hemifacial microsomia is characterized by unilateral facial hypoplasia, often with unilateral shortening of the mandible and subsequent malocclusion. Patients with hemifacial microsomia and facial asymmetry have a vertically short maxilla, tilted occlusal plane, and short mandible. Early treatment is necessary to avoid subsequent impaired midfacial growth. The standard treatment of these malformations consists of the application of bone grafts, which can lead to unpredictable growth. The new bone-lengthening procedure represents a limited surgical intervention and opens up a new perspective for treatment, especially in younger children with severe deformities. This report describes a case of hemifacial microsomia (Type-II left-sided hemifacial microsomia). The patient, a 10-year-old child, visited our clinic for facial asymmetry correction. He had a hypoplastic mandible, displaced ear lobe, 10 mm canting on the right side, and malocclusion. We planned DO to lengthen the left mandible in conjunction with a Le Fort I osteotomy for decanting and then perform a right intraoral vertical ramus osteotomy (IVRO). Progressive distraction at a rate of 0.5 mm/12 hours was initiated 7 days postoperatively. The duration of DO was 17 days. The consolidation period was 3 months. Satisfactory results were obtained in our case, indicating that DO can be used successfully for functional, aesthetic reconstruction of the mandible. We report a case involving DO in conjunction with orthognathic surgery for correcting mandibular hypoplasia with a review of the literature.
Bony Callus ; Child ; Cleft Lip ; Congenital Abnormalities ; Dental Occlusion ; Ear ; Facial Asymmetry ; Goldenhar Syndrome* ; Humans ; Malocclusion ; Mandible ; Maxilla ; Orthognathic Surgery ; Osteogenesis ; Osteogenesis, Distraction* ; Osteotomy ; Palate ; Retrognathia ; Transplants

Recently, laser treatment of benign prostatic hyperplasia (BPH) is considered as a promising alternative to traditional transurethral resection of the prostate (TURP). To evaluate the effectiveness and safety of laser therapy on BPH, we compared the results of transurethral balloon laser thermotherapy (TUBALT, n=13) and Hybrid laser prostatectomy (HLP, n=21) with those of TURP (n=25) in 58 patients with mild and moderate BPH. Following data were evaluated at postoperative 1, 3 and 6 months : AUA symptom score (SS), maximal flow rate (Qmax), subjective symptom improvement (SI), postoperative complications. All 3 groups show significant improvement after treatment in the Qmax values. Among 3 groups, the Qmax value was lower in TUBALT group (12.9+/-3.3 ml/sec) than those in HLP group (15.5+/-5.2 ml/sec) and TURP group (18.7+/-5.3 ml/sec) on postoperative 6 months. The Qmax values were not significantly different between HLP and TURP groups. In the SS values, all 3 groups show significant improvement after treatment and, TUBALT (9.9+/-9.7) and HLP (10.3+/-9.4) group were comparable to TURP group (5.2+/-4.2) on postoperative 6 months. In global assessment of SI, both HLP (87.5%) and TUBALT (75%) group were also comparable to TURP (90%) group on postoperative 3 months. but TUBALT group showed delayed symptom improvement compared to TURP group. Postoperative complications were minimal both in HLP and TUBALT groups, compared to TURP group. These results suggest that both HLP and TUBALT are effective in mild and moderate BPH, Further more, HLP treatment could be considered a promising alternative to TURP.
Humans ; Hyperthermia, Induced* ; Laser Therapy ; Postoperative Complications ; Prostate ; Prostatectomy* ; Prostatic Hyperplasia* ; Transurethral Resection of Prostate

To identify regulatory molecules which play key roles in the development of obesity, we investigated the transcriptional profiles in 3T3-L1 cells at early stage of differentiation and analyzed the promoter sequences of differentially regulated genes. One hundred and sixty-one (161) genes were found to have significant changes in expression at the 2nd day following treatment with differentiation cocktail. Among them, 86 transcripts were up-regulated and 75 transcripts were down-regulated. The 161 transcripts were classified into 10 categories according to their functional roles; cytoskeleton, cell adhesion, immune, defense response, metabolism, protein modification, protein metabolism, regulation of transcription, signal transduction and transporter. To identify transcription factors likely involved in regulating these differentially expressed genes, we analyzed the promoter sequences of up- or -down regulated genes for the presence of transcription factor binding sites (TFBSs). Based on coincidence of regulatory sites, we have identified candidate transcription factors (TFs), which include those previously known to be involved in adipogenesis (CREB, OCT-1 and c-Myc). Among them, c-Myc was also identified by our microarray data. Our approach to take advantage of the resource of the human genome sequences and the results from our microarray experiments should be validated by further studies of promoter occupancy and TF perturbation.
3T3-L1 Cells* ; Adipogenesis* ; Binding Sites ; Cell Adhesion ; Cytoskeleton ; Gene Expression Profiling* ; Genome, Human ; Humans ; Metabolism ; Microarray Analysis ; Obesity ; Signal Transduction ; Transcription Factors ; Transcriptome*

Cystic fibrosis (CF) is a chronic progressive autosomal recessive disease and caused by cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations. CFTR gene encodes an ion channel involved in salt and water transport in multiple organs including lung, pancreas and reproductive tract. To date, more than 2,000 mutations of CFTR gene have been identified. Because the age of onset and the severity of symptoms may vary greatly, a diagnosis of CF can be challenging. Although CF is common in Caucasians, it is extremely rare in Koreans. A few cases of CF have been reported in Korean children since 1988. Here, we report a 13-year-old girl carrying c.263T>G (p.Leu88X) and c.2977G>T (p.Asp993Tyr). Furthermore, we reviewed 22 Korean cystic fibrosis cases reported in the literature.