No abstract available.
Down Syndrome* ; Myeloproliferative Disorders*

Myasthenia gravis and autoimmune thyroid disorders often overlap. It is known that hyperthyroidism occurs in 2~17.5% of patients with myasthenia gravis. Thyrotoxicosis may influence the clinical course of myasthenia gravis. Overlapping clinical features may cause diagnostic confusion when Graves' disease and myasthenia gravis co-exist. Thus, various tests may be needed to distinguish these two diseases. It is clinically important to screen patients with myasthenia gravis for the co-existence of autoimmune thyroid disorders and vice versa. We cared for a patient with Graves' disease associated with ocular myasthenia gravis who presented with fluctuating double vision and ptosis. Ocular myasthenia gravis was diagnosed by electrophysiologic study and presence of acetylcholine receptor antibody. The patient had a favorable clinical and laboratory response to treatment with an anticholinesterase (pyridostigmine) and an antithyroid drug (propylthiouracil), and he had minimal symptoms at the 9-month follow-up examination.
Acetylcholine ; Diplopia ; Follow-Up Studies ; Graves Disease ; Humans ; Hyperthyroidism ; Myasthenia Gravis ; Thyroid Gland ; Thyrotoxicosis

BACKGROUND/AIMS: New definitions of acute kidney injury (AKI) have recently emerged. Some studies have suggested that duration of AKI is an additional predictive parameter for mortality. Here, we evaluated whether AKI duration was predictive of long-term mortality in patients with hospital-acquired acute kidney injury (HAAKI). METHODS: We prospectively enrolled patients who developed HAAKI at an urban university hospital, from September 2007 to August 2008 and followed them until December 2011. Patients were divided into two groups by duration of the AKI (1 to 5 days vs. > or = 6 days), and long-term mortality was compared. RESULTS: HAAKI developed in 1.2% of patients during the enrollment period. The median follow-up period was 240 days (interquartile range, 53 to 1,428). In 42.3% of patients (n = 52), the AKI lasted 1 to 5 days, while it lasted > or = 6 days in 57.7% (n = 71). Survival analysis showed that a longer duration of AKI increased the risk of death. Long-term survival was significantly different in the two groups. CONCLUSIONS: The duration of AKI influenced mortality rates in hospitalized patients. Thus, AKI duration is a parameter affecting mortality in HAAKI.
Acute Kidney Injury/diagnosis/etiology/*mortality/therapy ; Aged ; Female ; *Hospitalization ; Hospitals, University ; Humans ; Kaplan-Meier Estimate ; Male ; Middle Aged ; Prognosis ; Proportional Hazards Models ; Prospective Studies ; Republic of Korea ; Risk Factors ; Time Factors

Although hypertension is a well-known risk factor for chronic kidney disease (CKD), the blood pressure (BP) at which antihypertensive interventions should be initiated remains to be determined. Therefore, we investigated the association between BP and CKD in treatment-naïve individuals. Methods: This prospective cohort study considered 7,343 individuals in the Korean Genome and Epidemiology Study who were not taking antihypertensive medications. Subjects were categorized into six groups according to their systolic BP (SBP) and five groups according to their diastolic BP (DBP). The primary outcome was incident CKD, which was defined as an estimated glomerular filtration rate of <60 mL/min/1.73 m2 or the development of proteinuria. The secondary outcome was incident cardiovascular disease (CVD). Results: In the time-varying Cox models, the hazard ratios (95% confidence interval [CI]) for CKD were 1.39 (1.10–1.77) with SBP 130–139 mmHg, 1.79 (1.40–2.28) with SBP 140–159 mmHg, and 3.22 (2.35–4.40) with SBP ≥ 160 mmHg, compared with SBP 100–119 mmHg. In addition, the hazard ratios (95% CI) for CKD were 1.88 (1.48–2.37) with DBP 90–99 mmHg and 4.30 (3.20– 5.76) with DBP ≥ 100 mmHg, compared with DBP 70–79 mmHg. A significantly increased CVD risk was also observed in subjects with SBP ≥ 130 mmHg or DBP ≥ 90 mmHg. Conclusion: Our findings indicate that SBP ≥ 130 mmHg and DBP ≥ 90 mmHg are associated with an increased risk of CKD. Therefore, BP-lowering strategies should be considered starting at those thresholds to prevent CKD development.

Background: Foot deformities can cause abnormal biomechanics of the ankle joint and the development of osteoarthritis. It was hypothesized that foot deformities would be related to medial ankle osteoarthritis, and this study investigated this relationship using radiographic measurements. Methods: Seventy-six ankles of 76 patients (32 men and 44 women; mean age, 69.0 years) with medial ankle osteoarthritis were included. Eleven radiographic measurements evaluated ankle joint orientation (tibial plafond inclination [TPI], medial distal tibial angle [MDTA], and anterior distal tibial angle [ADTA]), ankle joint incongruency (tibiotalar tilt [TT]), foot deformities (lateral talofirst metatarsal angle [Lat talo-1MT], anteroposterior talo-first metatarsal angle [AP talo-1MT], and talonavicular coverage), talar body migration (medial talar center migration [MTCM] and anterior talar center migration [ATCM]), internal rotation (IR) of the talus, and mechanical tibiofemoral angle. All were statistically analyzed using Pearson’s correlation coefficients and regression analyses. Results: Ankle joint orientation to the ground (TPI, p = 0.002), increased foot arch (Lat talo-1MT, p < 0.001), and IR of the talus (p = 0.001) were significantly associated with ankle joint incongruency (TT) in linear regression analysis. Ankle joint incongruency (TT, p = 0.003), medial talar body migration (MTCM, p = 0.042), and increased foot arch (Lat talo-1MT, p = 0.022) were significantly associated with IR of the talus in the binary logistic regression analysis. MTCM was significantly correlated with TPI (r = 0.251, p = 0.029), TT (r = 0.269, p = 0.019), MDTA (r = 0.359, p = 0.001), ATCM (r = –0.522, p < 0.001), and AP talo-1MT (r = 0.296, p = 0.015). ATCM was significantly correlated with TPI (r = –0.253, p = 0.027), ADTA (r = 0.349, p = 0.002), and Lat talo-1MT (r = –0.344, p = 0.002). Conclusions Ankle joint orientation, foot deformities, and talar rotation were associated with ankle joint incongruency in medial ankle osteoarthritis when evaluated radiographically. These findings need to be considered during surgical treatment for medial ankle osteoarthritis. However, the biomechanical significance of these radiographic measurements requires further investigation.

An increased pericoronary fat attenuation index (FAI) on computed tomography angiography (CTA) is associated with increased all-cause and cardiac mortality in the general population. However, the ability of pericoronary FAI to predict long-term outcomes in chronic kidney disease (CKD) patients is unknown. Methods: In this single-center retrospective longitudinal cohort study, we assessed the utility of CTA-based pericoronary FAI measurement to predict mortality of CKD patients, including those with end-stage renal disease (ESRD). Mapping and analysis of pericoronary FAI involved three major proximal coronary arteries. The prognostic value of pericoronary FAI for long-term mortality was assessed with multivariable Cox regression models. Results: Among 268 CKD participants who underwent coronary CTA, 209 participants with left anterior descending artery (LAD) FAI measurements were included. The pericoronary FAI measured at the LAD was not significantly associated with adjusted risk of allcause mortality (hazard ratio [HR], 2.08; 95% confidence interval [CI], 0.94–3.51) in any CKD group. However, ESRD patients with elevated pericoronary FAI values had a greater adjusted risk of all-cause mortality compared with the low-FAI group (HR, 2.26; 95% CI, 1.11–4.61). Conclusion: The pericoronary FAI measured at the LAD predicted long-term mortality in patients with ESRD, which could provide an opportunity for early primary intervention in ESRD patients.

PURPOSE: We compared the clinical outcomes of allogeneic bone marrow transplantation (BM), peripheral blood stem cell transplantation (PB) and cord blood stem cell transplantation (CB) in children with malignant and non-malignant diseases. METHODS: We retrospectively analysed the engraftment speed, episodes of infection, acute graft versus host disease (GVHD), and survival rate in 27 children who underwent hematopoietic stem cell transplantation (HSCT) at Dong-A Cancer Center from August 1998 to July 2001. RESULTS: HSCT were done with BM in 16 patients, CB in 6 and PB in 5. The neutrophil and platelet engraftment were achieved at 13.27+/-4.10, 24.58+/-9.41 days in BM, 12.00+/-1.09, 15.88+/-4.42 days in PB, and 39.00+/-15.68, 76.50+/-37.01 days in CB (P=0.001, P=0.001). There were 17 episodes of bacteremia and 10 episodes of viral infections without any significant differences between stem cell sources. There were 8 cases (7 in BM, 1 in CB) of acute GVHD, 4 cases (2 in BM, 2 in CB) of graft failure and 3 cases of relapse (1 in BM, 2 in CB) after HSCT. The duration of median follow-up was 14.34+/-8.32 months in BM, 11.43+/-10.03 months in PB, and 16.56+/-13.76 months in CB. The overall and event free survival rate were 81.5% and 63.0%, respectively. CONCLUSION: There were no significant differences in episodes of infection between the types of HSCT. Although there were HLA mismatched donors for CB, the incidence of acute GVHD was lower, and graft failure or relapse rate was higher than BM.
Bacteremia ; Blood Platelets ; Bone Marrow Transplantation ; Child* ; Cord Blood Stem Cell Transplantation ; Disease-Free Survival ; Follow-Up Studies ; Graft vs Host Disease ; Hematologic Diseases* ; Hematopoietic Stem Cell Transplantation* ; Hematopoietic Stem Cells* ; Humans ; Incidence ; Neutrophils ; Peripheral Blood Stem Cell Transplantation ; Recurrence ; Retrospective Studies ; Stem Cells* ; Survival Rate ; Tissue Donors ; Transplants

Background: Metformin has recently been shown not to increase the risk of lactic acidosis in patients with chronic kidney disease (CKD). Thus, the criteria for metformin use in this population has expanded. However, the relationship between metformin use and clinical outcomes in CKD remains controversial. Methods: This study considered data from 97,713 diabetes patients with an estimated glomerular filtration rate of <60 mL/min/1.73 m2. The primary outcome was major adverse cardiac and cerebrovascular events (MACCE), and the secondary outcomes were all-cause mortality and incident end-stage renal disease (ESRD). Results: Metformin users had a significantly higher risk of MACCE than non-users (hazard ratio [HR], 1.20; 95% confidence interval [CI], 1.14–1.26; p < 0.001). However, metformin users had a lower risk of all-cause mortality (HR, 0.78; 95% CI, 0.74–0.81; p < 0.001) and ESRD (HR, 0.44; 95% CI, 0.42–0.47; p < 0.001) during follow-up than non-users did. The relationships between metformin use and clinical outcomes remained consistent in propensity score matching analyses and subgroup analyses of patients with adequate adherence to anti-diabetes medication. Conclusion Treatment with metformin was associated with an increased risk of MACCE in patients with diabetes and CKD. However, metformin users had a lower risk of all-cause mortality and ESRD during follow-up than non-users did. Therefore, metformin needs to be carefully used in patients with CKD.

BK virus nephropathy has emerged as an important cause of renal allograft dysfunction. Reduction in immunosuppression is the mainstay of BK virus nephropathy treatment. However, decreasing immunosuppressive medications is not sufficient for treatment of BK virus nephropathy. Therefore, there is a need for other treatment strategies such as cidofovir, leflunomide, and intravenous immunoglobulin in combination with immunosuppression reduction. Ciprofloxacin has recently been reported to have antiviral activity and decrease BK viral load in kidney transplant recipients. These findings suggest that the use of ciprofloxacin represents a valuable treatment strategy in patients with BK virus nephropathy. Here, we report on our experience with three patients who developed presumptive BK virus nephropathy after kidney transplantation, who, after 2 months of ciprofloxacin treatment, showed disappearance of BK viremia and improvement in the estimated glomerular filtration rate. Ciprofloxacin may be considered an effective treatment option for BK viremia in kidney transplant recipients.
Allografts ; BK Virus ; Ciprofloxacin ; Glomerular Filtration Rate ; Humans ; Immunoglobulins ; Immunosuppression ; Kidney Transplantation ; Kidney* ; Transplantation* ; Viral Load ; Viremia

Antibody-mediated rejection (ABMR) is associated with poor renal allograft survival. It shows poor response to conventional treatment with plasmapheresis, rituximab, and intravenous immunoglobulin. Bortezomib, a proteasome inhibitor used for treatment of multiple myeloma, has recently been reported as a treatment alternative for recipient desensitization and ABMR. A 58-year-old man was diagnosed with mixed-type ABMR with donor specific antibodies and acute T cell-mediated rejection early after kidney transplantation. Conventional therapy was administered, including antithymocyte globulin, plasmapheresis, and rituximab; however, his condition was found to be refractory to these antihumoral therapies. Following administration of bortezomib, his serum creatinine level returned to baseline with stable graft function. His serum creatinine level remains stable at 1.3 mg/dL at 10 months posttransplantation. Bortezomib is effective for treatment of refractory ABMR following kidney transplantation.
Allografts ; Antibodies ; Antilymphocyte Serum ; Bortezomib ; Creatinine ; Humans ; Immunoglobulins ; Kidney Transplantation ; Kidney* ; Middle Aged ; Multiple Myeloma ; Plasmapheresis ; Proteasome Inhibitors ; Rituximab ; Tissue Donors ; Transplantation* ; Transplants