No abstract available.
Fracture Fixation, Intramedullary*

Spinal muscular atrophy(SMA) is a genetic disorder of the motor neurons that cause muscular weakness and muscular atrophy due to anterior horn cell degeneration. Classic spinal muscular atrophy patient is caused by mutation in the chromosome 5(q11.2-q13.3), and the majority of the patient shows homozygous deletion of the telomeric survival motor neuron(SMN) gene in the chromosome 5. Deletion of exon 7 and 8 of the SMN gene and deletion of exon 4 and 5 of the neuronal apoptosis inhibitory protein(NAIP) are typically observed in SMA patients. The SMN protein plays a role in an essential cell metabolism process, the splicing of pre mRNA in the spliceosomes. We report a 7 month old male with SMA. He showed rapidly aggrdvatial muscular weakness and died at 7 months. His DNA analysis proved deletion of exon 7 and 8 of the telomeric copy of the SMN gene.
Anterior Horn Cells ; Apoptosis ; Chromosomes, Human, Pair 5 ; DNA ; Exons* ; Humans ; Infant ; Male ; Metabolism ; Motor Neurons* ; Muscle Weakness ; Muscular Atrophy ; Muscular Atrophy, Spinal* ; Neurons ; RNA Precursors ; Spliceosomes

PURPOSE: To assess the clinical reliability of the Bosniak IIF category and to determine the proper radiologic follow-up duration and intervals for category IIF complex renal cysts. MATERIALS AND METHODS: We studied 201 patients with category IIF renal cysts from January 1996 to January 2011. Renal cyst progression to category III was defined as an increase in complexity of the cyst in follow-up radiologic studies. We monitored radiologic changes and progression of renal cysts during the follow-up period and analyzed the pathologic results of those patients who were treated surgically. RESULTS: At a mean follow-up of 20 months, only 14 cases (7%) showed evidence of progression to stage III, with a mean time to progression of 11 months (range, 3 to 65 months). There were no significant differences in age, gender, cyst size, or change in cyst size between the progressive and non-progressive groups. Of 12 cases treated surgically, 10 cases (83.3%) showed renal cell carcinoma with pT1 stage, and there was no recurrence during postoperative follow-up of 23 months. Of the 187 patients without radiologic progression, 23 cases were treated surgically, and all of them showed benign cysts. CONCLUSIONS: The IIF category showed significant clinical reliability by a low rate of radiologic progression and a high rate of malignancy in the radiologic progressive group but a low rate of malignancy in the non-progressive group. Although it is hard to decide on a proper follow-up duration because of the variable time to progression, too frequent follow-up study seems to be unnecessary considering that most malignant cases were of a low stage.
Carcinoma, Renal Cell ; Disease Progression ; Follow-Up Studies ; Humans ; Kidney ; Recurrence

Acute phlegmonous infection of the gastrointestinal tract is characterized by purulent inflammation of the submucosa and muscular layer with sparing of the mucosa. The authors report a rare case of acute diffuse phlegmonous esophagogastritis, which was well diagnosed based on the typical chest computed tomographic (CT) findings and was successfully treated. A 48-yr-old man presented with left chest pain and dyspnea for three days. Chest radiograph on admission showed mediastinal widening and bilateral pleural effusion. The patient became febrile and the amount of left pleural effusion is increased on follow-up chest radiograph. Left closed thoracostomy was performed with pus drainage. A CT diagnosis of acute phlegmonous esophagogastritis was suggested and a surgery was decided due to worsening of clinical condition of the patient and radiologic findings. Esophageal myotomies were performed and the submucosal layer was filled with thick, cheesy materials. The patient was successfully discharged with no postoperative complication.
Acute Disease ; Cellulitis/complications/*diagnosis/radiography ; Drainage ; Esophagitis/complications/*diagnosis/surgery ; Gastritis/complications/*diagnosis/surgery ; Humans ; Klebsiella Infections/diagnosis ; Klebsiella pneumoniae/isolation & purification ; Male ; Middle Aged ; Pleural Effusion/etiology/radiography ; Thoracostomy ; Tomography, X-Ray Computed

PURPOSE: To determine the accuracy of magnetic resonance (MR) arthrography in the detection and staging of lesions of the acetabular labrum. MATERIALS AND METHODS: Fifteen hips of 14 patients with chronic hip pain and clinical suspicion of labral lesions were examined with direct or indirect MR arthrography and underwent subsequent arthroscopic evaluation. The study population consisted of six women and eight men aged between 40 and 59 years. Nine arthrograms were obtained intra-articular administration of gadolinium solution, and six involved articular motion exercise after intravenous administration of gadolinium solution. In 14 cases a phased-array pelvic coil was used, and a shoulder coil in one. Fat-suppressed T1-weighted images were obtained in the coronal, oblique coronal and oblique axial planes, and T1-and T2-weighted images were obtained in the axial plane. Labral lesions were graded according to the Czerny classification and evaluated on the basis of presence or absence of a tear and location (anteroinferior, anterosuperior, superior, posterosuperior, posteroinferior). The findings were then correlated with the arthroscopic findings. RESULTS: After direct and indirect MR arthography, the findings-based on the Czerny classification -were as follows : stage IA:3; IB:1; IIA:8; IIB:1; IIIA:4; IIIB:0. MR arthrography also showed that seven cases were located in the anterosuperior portion of the labrum, seven in the superior portion, and three in the posteroinferior portion. The sensitivity, specificity, positive predictive value, negative predictive value, and accuracy of MR arthrography for diagnosis of the labral tear was 92%, 75%, 92%, 75 %, and 88 %, respectively. CONCLUSION: In this preliminary study, MR arthrography appears to be a promising imaging modality for accurate diagnosis and useful for screening patients with chronic hip pain.
Acetabulum* ; Administration, Intravenous ; Arthrography* ; Classification ; Diagnosis* ; Female ; Gadolinium ; Hip ; Humans ; Male ; Mass Screening ; Sensitivity and Specificity ; Shoulder

BACKGROUND: Helicobacter pylori colonizes the gastric surface epithelium and the mucus gel layer. It has been known that H. pylori infection decreased the gastric mucin expression. The aim of this study was to determine the effect of H. pylori eradication on mucin expression (MUC5AC, MUC6 and MUC1) in the gastric epithelium. METHODS: This study included 20 patients positive for H. pylori whom successful eradication was performed between March 1998 and December 1999. H. pylori status was determined by histology, rapid urase test and urea breath test. Gastric antral biopsy specimens were examined by immunohistochemistry for mucin (MUC5AC, MUC6 and MUC1) expression. The distribution of epithelial cells expressing MUC5AC was calculated at two sites (surface mucous cells, pyloric glands). Two scores system (weak-strong) was used to assess staining intensity. RESULTS: There was a gradient of MUC5AC expression, higher to lower from the surface to the glands. Increased MUC5AC expression in the surface mucous cell (p=0.013) and in the glands (p=0.008) was found after H. pylori eradication. MUC6 and MUC1 distribution was not changed after H. pylori eradication. CONCLUSION: MUC5AC expression was increased after H. pylori eradication. These results suggest that MUC5AC may relate in the pathogenesis of H. pylori.
Biopsy ; Breath Tests ; Colon ; Epithelial Cells ; Epithelium ; Gastric Mucins ; Helicobacter pylori* ; Helicobacter* ; Humans ; Immunohistochemistry ; Mucins* ; Mucus ; Urea

Background: The Korean Society of Nephrology (KSN) has maintained a nationwide end-stage renal disease (ESRD) registry data from Korean Renal Data System (KORDS) since 1985, as the representative registry of ESRD patients in Korea. This review is aimed to update the status of domestic ESRD and to provide evidence on the direction of dialysis therapy. Methods: The KORDS Committee of KSN has collected data on dialysis centers and patients through an online registry program, and the data from 1986 to 2019 were analyzed. Results: The incidence and prevalence of ESRD patients in Korea are increasing. The ESRD population numbered more than 100,000 in 2019, doubling during the 10 years since 2010. The proportion of diabetes mellitus as a major cause of ESRD seems to have reached a plateau. The increasing number of elderly dialysis patients is a constant trend, with more than half for the proportion of patients older than 65 years old in 2019. All-cause mortality decreased for the last approximately 20 years, regardless of sex, age, and cause of ESRD. The 5-year patient survival rate in both hemodialysis and peritoneal dialysis increased from 2001 to 2013. Since 2013, the patient survival rates in peritoneal dialysis were similar to those in hemodialysis. Cardiovascular complications were the leading cause of death in ESRD patients. Conclusions The incidence and prevalence of Korean ESRD patients have increased over time, although patient survival has also steadily increased. The establishment of a surveillance method to address the major cause of mortality in ESRD patients will help improve outcomes.

Background: The Korean Society of Nephrology (KSN) has maintained a nationwide end-stage renal disease (ESRD) registry data from Korean Renal Data System (KORDS) since 1985, as the representative registry of ESRD patients in Korea. This review is aimed to update the status of domestic ESRD and to provide evidence on the direction of dialysis therapy. Methods: The KORDS Committee of KSN has collected data on dialysis centers and patients through an online registry program, and the data from 1986 to 2019 were analyzed. Results: The incidence and prevalence of ESRD patients in Korea are increasing. The ESRD population numbered more than 100,000 in 2019, doubling during the 10 years since 2010. The proportion of diabetes mellitus as a major cause of ESRD seems to have reached a plateau. The increasing number of elderly dialysis patients is a constant trend, with more than half for the proportion of patients older than 65 years old in 2019. All-cause mortality decreased for the last approximately 20 years, regardless of sex, age, and cause of ESRD. The 5-year patient survival rate in both hemodialysis and peritoneal dialysis increased from 2001 to 2013. Since 2013, the patient survival rates in peritoneal dialysis were similar to those in hemodialysis. Cardiovascular complications were the leading cause of death in ESRD patients. Conclusions The incidence and prevalence of Korean ESRD patients have increased over time, although patient survival has also steadily increased. The establishment of a surveillance method to address the major cause of mortality in ESRD patients will help improve outcomes.

Korean Renal Data System (KORDS) is a nationwide end-stage renal disease (ESRD) registry database operated by the Korean Society of Nephrology (KSN). Diabetes mellitus is currently the leading cause of ESRD in Korea; this article provides an update on the trends and characteristics of diabetic ESRD patients. The KORDS Committee of KSN collects data on dialysis centers and patients through an online registry program. Here, we analyzed the status and trends in characteristics of diabetic chronic kidney disease stage 5D (CKD 5D) patients using data from 2001 to 2021. In 2021, the dialysis adequacy of hemodialysis (HD) was lower in diabetic CKD 5D patients than in nondiabetic CKD 5D patients, while that of peritoneal dialysis (PD) was similar. Diabetic CKD 5D patients had a higher proportion of cardiac and vascular diseases and were more frequently admitted to hospitals than nondiabetic CKD 5D patients, and the leading cause of death was cardiac disease. From 2001 to 2020, diabetic CKD 5D patients had a higher mortality rate than nondiabetic CKD 5D patients, but in 2021 this trend was reversed. Diabetic PD patients had the highest mortality rate over 20 years. The mortality rate of diabetic HD patients was higher than that of nondiabetic HD patients until 2019 but became lower starting in 2020. There was a decreasing trend in mortality rate in diabetic CKD 5D patients, but cardiac and vascular diseases were still prevalent in diabetic CKD 5D patients with frequent admissions to hospitals. More specialized care is needed to improve the clinical outcomes of diabetic CKD 5D patients.

With an increasing aging population, the mean age of patients with end-stage kidney disease (ESKD) is globally increasing. However, the current clinical status of elderly patients undergoing hemodialysis (HD) is rarely reported in Korea. The current study analyzed the clinical features and trends of older patients undergoing HD from the Korean Renal Data System (KORDS) database. The patients were divided into three groups according to age: <65 years (the young group), n = 50,591 (35.9%); 65–74 years (the younger-old group), n = 37,525 (26.6%); and ≥75 years (the older-old group), n = 52,856 (37.5%). The proportion of older-old group undergoing HD significantly increased in incidence and decreased in prevalence from 2013 to 2022. The median levels of hemoglobin, serum creatinine, albumin, calcium, phosphorus, and intact parathyroid hormone significantly decreased in the older-old group. The proportions of arteriovenous fistula creation and left forearm placement showed decreased trends with age. Although the utilization of low surface area dialyzers increased with age, the dialysis adequacy, including urea reduction ratio and Kt/V was within acceptable range in the older-old group on HD. Over the past 20 years, the mortality rate in the older-old group has increased, with cardiovascular diseases decreasing and infectious diseases increasing. The incidence of elderly patients undergoing HD has increased over time, but the high mortality of the older-old group needs to be solved. Therefore, it is imperative to develop holistic strategies based on age and individual needs for patients with ESKD.