BACKGROUND: Retroperitoneal fibrosis (RPF) is a rare disease characterized by fibroinflammatory tissue in the periaortic or periiliac retroperitoneum, where it frequently encases ureters. There is emerging evidence that a subset of this disease is part of a spectrum of multisystemic autoimmune diseases collectively referred to as “immunoglobulin G4 (IgG4)-related disease”. METHODS: We retrospectively analyzed 27 idiopathic RPF patients and identified a subset as IgG4-related RPF, which we categorized according to recently published comprehensive diagnostic criteria. We compared clinical and laboratory characteristics and response to treatment between the two groups. RESULTS: Of 27 total patients, 16 (59.3%) were diagnosed as having IgG4-related RPF, and these were predominantly male. They were also significantly older and more likely to have other organ involvement, hydronephrosis, and postrenal acute kidney injury (AKI) compared to those with idiopathic RPF. However, there was no difference in response rate to systemic steroid treatment. CONCLUSION: IgG4-related RPF accounts for a substantial portion of RPF cases previously identified as “idiopathic RPF” in Korea. Clinical and laboratory characteristics of IgG4-related RPF are similar to those of idiopathic RPF except for a striking male predominance, older age, and higher incidence of postrenal AKI in IgG4-related RPF. More comprehensive, prospective studies are needed to clearly distinguish IgG4-related RPF from idiopathic RPF based on clinical manifestation and to further assess treatment response and long-term prognosis.
Acute Kidney Injury ; Autoimmune Diseases ; Humans ; Hydronephrosis ; Immunoglobulins ; Incidence ; Korea ; Male ; Prognosis ; Prospective Studies ; Rare Diseases ; Retroperitoneal Fibrosis ; Retrospective Studies ; Strikes, Employee ; Ureter

PURPOSE: To investigate the influence of the change in medial rectus resection amount caused by traction with muscle hooks on surgical results in exotropia. METHODS: In this prospective study 38 exotropia patients underwent unilateral lateral rectus recession-medial rectus resection (R & R). After isolation of the medial rectus muscle, the length of the muscle was measured with a muscle hook, and the portion to be resected was marked using gentian violet stain with calipers. After the medial rectus was retracted with two muscle hooks in either direction, its length was measured again with the calipers. Group 1 includes the patients with the planned resection amount changed to a more 0.5-1.0 mm when the rectus muscle was retracted, while group 2 did not change with the second muscle hook. The surgical results were compared between the 2 groups at postoperative 6 months. RESULTS: Mean resection amount was changed from 5.07 +/- 0.76 mm to 5.36 +/- 0.73 mm after pulling the second muscle hook. Sixteen patients (42.1%) were included in group 1 and 22 patients (57.9%) in group 2. There was no significant statistical difference in mean preoperative and postoperative deviated angle between the two groups. Success rates defined as the ocular alignment within +/- 10 PD in the primary position at postoperative 6 months were 75.0% in group 1 and 86.3% in group 2, which is not stastistically different (p > 0.05). CONCLUSIONS: Medial rectus resection amounts can be changed with the muscle traction with two muscle hooks, which induced the planned amount of resection less than first measured resection amount. However, there was little influence on surgical outcomes from the change occurred to a maximum 1 mm in resection measurement due to traction with two muscle hooks.
Exotropia ; Gentian Violet ; Humans ; Muscles ; Prospective Studies ; Strabismus ; Traction

PURPOSE: Organophosphate insecticide poisoning is common in Korea, but there is no definitive guideline for determining the severity of the poisoning and the predictive factors. Therefore, we evaluated the organophosphate poisoned patients and we divided them into two groups, the survivors and the dead, and the results might be useful for treating organophosphate poisoning patients. METHODS: We performed a retrospective analysis of 68 organophosphate poisoned patients who visited the Chosun University Hospital Emergency Medical Center during a 24-month period from January, 2007 to December, 2008. We made a work sheet of the patients' characteristics and the collected data was analyzed and we compared this data between the survivor group and the dead patient group. RESULTS: There were significant differences between the survivor group and the dead patient group for the mean age, the alcohol intake state and the typically expressed signs. The dead patients had lower blood pressure, tachycardia and a lower Glasgo Coma Score (GCS) score than the survivor group. On the arterial blood gas analysis, the dead patients had more severe acidemia and they had lower saturations. Increased serum amylase levels were found in the dead patients. The survivors' initial and follow up serum pseudocholinesterase activity (after 6~8 days) was significantly higher than that of the dead group. The total amount of atropine injected to patient was less in the survivors than that in the dead patients. CONCLUSION: Old age and expressing the typical intoxication signs, a lower GCS score and blood pressure, showing acidosis on the gas analysis and low serum cholinesterase activity may be useful as poor prognostic indicators for patients with organophosphate poisoning. We suggest that physicians must pay careful attention to the signs and prognostic factors of organophosphate insecticide poisoned patients.
Acidosis ; Amylases ; Atropine ; Blood Gas Analysis ; Blood Pressure ; Cholinesterases ; Coma ; Emergencies ; Follow-Up Studies ; Humans ; Korea ; Organophosphate Poisoning ; Pseudocholinesterase ; Retrospective Studies ; Survivors ; Tachycardia

PURPOSE: To evaluate changes in muscle length when retracting the extraocular rectus muscle with muscle hooks during strabismus surgery. METHODS: Forty-four rectus muscles of 42 patients consecutively resected in two hospitals (A, B) were included in this study. After isolation of the rectus muscle, the length of the muscle was recorded and the portion to be resected was marked using gentian violet stain on the tip of calipers. After the rectus muscle was retracted with two muscle hooks in either direction, its length was measured again with the calipers. RESULTS: The length of the rectus muscle was not changed by retraction in 25 of 44 muscles (56.8%). The length of the muscle was changed by 0.5 mm in 13 muscles (29.5%) and by 1mm in six muscles (13.6%). Changes of rectus muscle length over 0.5 mm were observed in 15 of 27 muscles of patients treated at hospital A (55.5%) and four of 17 muscles of patients treated at hospital B (23.5%). The results for the two hospitals were significantly different (p=0.037). CONCLUSIONS: Small errors in measurement can occur when muscle resections are performed during strabismus surgery. Institutional or clinician related factors may explain variations in error rates between hospitals.
Gentian Violet ; Humans ; Muscles ; Strabismus ; Traction

BACKGOUND: Although the incidence has decreased markedly, mortality from uremic pericarditis still remained high at 8-10% due to hemodynamic compromise. Moreover, it is difficult to diagnose and discriminate from other causes of pericarditis such as tuberculous pericarditis in its early stage. The aim of this study was to analyze the factors that were related to the development of uremic pericarditis and factors that could distinguish it from other causes of pericarditis. METHODS: Eighteen patients who received pericardiocentesis due to uremic pericarditis from 1996 to 2005 in Korea university hospital were enrolled. All patients were diagnosed as severe uremic pericarditis by echocardiography. And as a comparison group, 37 patients with tuberculous pericarditis and 20 patients with malignant pericarditis were also enrolled. Analysis of the factors that were related to the development of uremic pericarditis or comparison of clinical, biochemical factors in uremic, tuberculous or malignant pericarditis were also done. RESULTS: In uremic pericarditis, the proportion of patients with peritoneal dialysis was higher (55.6%). The amount of pericardial effusion showed a positive correlation with the duration of dialysis, whereas showed negative correlation with hemoglobin and cholesterol levels. Pericardial fluid ADA was significantly higher in tuberculous pericarditis and pericardial fluid glucose was higher in uremic pericarditis. No specific factors that were related to the development of pericardial tamponade were identified. CONCLUSION: The development of severe uremic pericarditis might be related to poor nutritional status. In the early stage, ADA and glucose levels in pericardial fluid could be useful in distinguishing uremic pericarditis from tuberculous pericarditis. Prospective studies that enroll large patient population can be helpful in identifying factors that are related to the development of uremic pericarditis or tamponade.
Cardiac Tamponade ; Cholesterol ; Dialysis ; Echocardiography ; Glucose ; Hemodynamics ; Humans ; Incidence ; Korea ; Mortality ; Nutritional Status ; Pericardial Effusion ; Pericardiocentesis ; Pericarditis* ; Pericarditis, Tuberculous ; Peritoneal Dialysis

Cryptococcus is an important opportunistic pathogen of fungal infection after organ transplantation. And, tuberculosis is also a major cause of infection in immunocompromised patients. We experienced a case of asymptomatic cryptococcal pulmonary infection detected by routine chest X-ray in a renal transplant patient, and a subsequent development of symptomatic multi-drug resistant pulmonary tuberculosis during oral fluconazole treatment. For the appropriate infection control, we should make the thorough evaluation in immunocompromised organ-transplant patients.
Cryptococcus* ; Fluconazole ; Humans ; Immunocompromised Host ; Infection Control ; Kidney Transplantation ; Organ Transplantation ; Thorax ; Transplants ; Tuberculosis* ; Tuberculosis, Pulmonary

BACKGROUND: Chronic systemic inflammation in ESRD patients due to uremia and hemodialysis procedure itself comes into notice as a main factor for premature mortality secondary to rapid progressing atherosclerosis. Various pro-inflammatory cytokine, known to mediate these reaction of malnutrition, inflammation and atherosclerosis, are regulated by anti-inflammatory cytokine, such as IL-10. Quantitative production of IL-10 shows interindividual variability determined genetically by polymorphisms of promotor gene. The aim of this study was to measure the degree of IL-10 synthesis in ESRD patients treated with hemodialysis and evaluate the association with genotypes and cardiovascular risk factors. METHODS: The IL-10 genotypes for polymorphic bases at position at -1082 was determined in 66 chronic hemodialysis patients and 98 healthy subjects using highly specific PCR and the lipopolysaccharide (LPS)-stimulated IL-10 (sIL-10) release from whole blood were measured by ELISA. RESULTS: The distribution of the IL-10 genotypes in hemodialysis patients were similar to the general population, but the proportion of A allele in hemodialysis group was significantly higher (72.3% vs 59.8%, p=0.05). sIL-10 concentration were lower in hemodialysis patients compared with normal control (21.1 pg/mg vs 36.1 pg/mg, p=0.001) and both groups showed same relationship of sIL-10 with genotypes, that AA type was low producer. In multiple regression analysis, sIL-10 of normal group correlated negatively with age, creatinine, uric acid and existence LVH, and positively with albumin, hemoglobin. On the other hand, lower albumin, lower ejection fraction on echocardiography and existence of left ventricular hypertrophy were associated with higher sIL-10 in hemodialysis group. CONCLUSION: Polymorphisms by IL-10 genotypes were associated with production of IL-10 by endotoxin stimulation, and sIL-10 was lower in hemodialysis patients than in normal control. According to relation of sIL-10 with cardiovascular risk factors such as existence LVH, ejection fraction and malnutrition, it could be suggested that sIL-10 is useful marker in evaluating the risk of cardiovascular events.
Alleles ; Atherosclerosis ; Cardiovascular Diseases ; Creatinine ; Echocardiography ; Enzyme-Linked Immunosorbent Assay ; Genotype* ; Hand ; Humans ; Hypertrophy, Left Ventricular ; Inflammation ; Interleukin-10* ; Kidney Failure, Chronic ; Malnutrition ; Mortality, Premature ; Polymerase Chain Reaction ; Renal Dialysis* ; Risk Factors* ; Uremia ; Uric Acid

Light chain deposition disease is caused by systemic paraprotein deposition resulting from monoclonal plasma cell dyscrasia. It is usually characterized rapidly progressive renal failure and multiple organ dysfunctions. Besides deposition of light chain, it can lead to multiple organ dysfunctions due to hyperviscosity syndrome. We experienced a case of 54-year-old man who presented as an acute renal failure, and elevation of liver enzyme. Radiologically, there was no abnormal finding except enlargement of both kidney in abdominal sonogram. Globulinuria was noticed on 24 hrs-urine study. The histologic findings of kidney showed lambda chain deposits in basement membrane of tubules and glomeruli, interstitium, and vessel walls. On 10th day of hospitalization, he developed sudden hypoxia that was not corrected by oxygen supplementation, and focal neurologic signs accompanied with a change of consciousness. We report a case of light chain deposition disease manifested as an acute renal failure and liver enzyme elevation with suspicious multiple organ embolic events later.
Acute Kidney Injury* ; Anoxia ; Basement Membrane ; Consciousness ; Hospitalization ; Humans ; Kidney ; Liver ; Middle Aged ; Neurologic Manifestations ; Oxygen ; Paraproteinemias ; Renal Insufficiency

Amyloidosis is known as a disease caused by the deposition of a insoluble and fibrous amyloid protein in the extracellular space of various organs and tissue. Intestinal amyloid deposition may develop motility disturbance, malabsorption, bleeding and perforation. A 70-year old woman with lower abdominal pain, watery diarrhea was admitted and had the past history of diabetes mellitus, hypertension for 8 years and rheumatoid arthritis for 10 year. On colonoscopic examination for evaluation of diarrhea, multiple edematous and shallow ulcers was found from distal sigmoid to terminal ileum. A green colored positive birifringent stained amorphous material was found in polarizing microscopy of colon biopsy specimen stained with Congo-red on microscopic examination,. We report a case of amyloidosis causing colon ulcers confirmed by colonoscopic biopsy with review literature.
Abdominal Pain ; Aged ; Amyloid* ; Amyloidosis ; Arthritis, Rheumatoid* ; Biopsy ; Colitis* ; Colon ; Colon, Sigmoid ; Diabetes Mellitus ; Diarrhea ; Extracellular Space ; Female ; Hemorrhage ; Humans ; Hypertension ; Ileum ; Microscopy ; Plaque, Amyloid ; Ulcer