ABSTRACT
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.
ABSTRACT
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.
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Evidence of the ethical appropriateness and clinical benefits of shared decision-making (SDM) are accumulating. This study aimed to not only identify physicians’ perspectives on SDM, and practices related to end-of-life care in particular, but also to gauge the effect of SDM education on physicians in Korea. Methods: A 14-item questionnaire survey using a modified Delphi process was delivered to nephrologists and internal medicine trainees at 17 university hospitals. Results: A total of 309 physicians completed the survey. Although respondents reported that 69.9% of their practical decisions were made using SDM, 59.9% reported that it is not being applied appropriately. Only 12.3% of respondents had received education on SDM as part of their training. The main obstacles to appropriate SDM were identified as lack of time (46.0%), educational materials and tools (29.4%), and education on SDM (24.3%). Although only a few respondents had received training on SDM, the proportion of those who thought they were using SDM appropriately in actual practice was high; the proportion of those who chose lack of time and education as factors that hindered the proper application of SDM was low. Conclusion: The majority of respondents believed that SDM was not being implemented properly in Korea, despite its use in actual practice. To improve the effectiveness of SDM in the Korean medical system, appropriate training programs and supplemental policies that guarantee sufficient application time are required.
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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.
ABSTRACT
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.
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Background@#Infection is the second leading cause of death in patients undergoing long-term dialysis. Peritoneal dialysis (PD) is associated with an increased risk of infection-related hospitalization (IRH) when compared with hemodialysis. In this study, we investigated the influence of IRH on clinical outcomes in incident PD patients. @*Methods@#In total, 583 incident PD patients were selected from the Clinical Research Center Registry for End-Stage Renal Disease, a nationwide multicenter prospective observational cohort study in Korea. Incident PD patients who had been hospitalized for infection-related diseases were defined as the IRH group. The primary outcome was all-cause mortality and the secondary outcome was technical failure. The median follow-up period was 29 months. @*Results@#Seventy-three PD patients (12.5%) were categorized in the IRH group. Multivariable logistic regression analysis showed that diabetes mellitus was a significant independent predictor for IRH (odds ratio, 2.43; 95% confidence interval [CI], 1.12 to 5.29; P = 0.007). The most common causes of IRH were peritonitis (63.0%) and respiratory tract infection (9.6%). Multivariable Cox proportional hazard model analysis showed that IRH was a significant independent risk factor for all-cause mortality (hazard ratio [HR], 2.51; 95% CI, 1.12 to 5.62; P = 0.026) and for the technical failure of PD (HR, 3.23; 95% CI, 1.90 to 5.51; P < 0.001). @*Conclusion@#Our data showed that after initiation of PD, IRH was significantly associated with higher risk of all-cause mortality and technical failure.
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Background@#Infection is the second leading cause of death in patients undergoing long-term dialysis. Peritoneal dialysis (PD) is associated with an increased risk of infection-related hospitalization (IRH) when compared with hemodialysis. In this study, we investigated the influence of IRH on clinical outcomes in incident PD patients. @*Methods@#In total, 583 incident PD patients were selected from the Clinical Research Center Registry for End-Stage Renal Disease, a nationwide multicenter prospective observational cohort study in Korea. Incident PD patients who had been hospitalized for infection-related diseases were defined as the IRH group. The primary outcome was all-cause mortality and the secondary outcome was technical failure. The median follow-up period was 29 months. @*Results@#Seventy-three PD patients (12.5%) were categorized in the IRH group. Multivariable logistic regression analysis showed that diabetes mellitus was a significant independent predictor for IRH (odds ratio, 2.43; 95% confidence interval [CI], 1.12 to 5.29; P = 0.007). The most common causes of IRH were peritonitis (63.0%) and respiratory tract infection (9.6%). Multivariable Cox proportional hazard model analysis showed that IRH was a significant independent risk factor for all-cause mortality (hazard ratio [HR], 2.51; 95% CI, 1.12 to 5.62; P = 0.026) and for the technical failure of PD (HR, 3.23; 95% CI, 1.90 to 5.51; P < 0.001). @*Conclusion@#Our data showed that after initiation of PD, IRH was significantly associated with higher risk of all-cause mortality and technical failure.
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Background/Aims@#Parathyroid hormone (PTH) is an important factor influencing immunologic dysfunction, but the effect of PTH level on infection-related outcomes remains unclear in incident dialysis. @*Methods@#We evaluated a multicenter prospective cohort study of 1,771 incident dialysis patients (1,260 hemodialysis and 511 peritoneal dialysis) in Korea. Patients were divided into three groups based on serum intact PTH (iPTH) level. The primary outcomes were all-cause and infection-related mortality and multivariate Cox regression analysis was performed to evaluate the role of iPTH in all-cause and infection-related mortality. @*Results@#During the follow-up period of 27.3 months, 175 patients (9.9%) died, and infection-related death represented 20% of all-cause mortality. Both all-cause mortality and infection-related mortality rates (p < 0.001 and p = 0.003, by logrank) were markedly higher in patients with serum iPTH < 150 pg/mL than in the other groups. Multivariate Cox regression analysis revealed that patients with serum iPTH < 150 pg/mL remained at higher risk for infection-related mortality than patients in the target range of 150 ≤ iPTH < 300 pg/mL, after adjusting for confounding variables (hazard ratio [HR], 2.52; 95% confidence interval, 1.06 to 5.99; p = 0.04). The HR of infection-related mortality in patients with serum iPTH < 150 pg/mL was significantly higher in patients with low serum phosphorus, low Ca × P product, low serum alkaline phosphatase and those older than 65 years. @*Conclusions@#Low serum iPTH level is an independent predictor of infection-related mortality in incident dialysis patients.
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BACKGROUND/AIMS@#Membranous nephropathy (MN) is the most common primary glomerular disease diagnosed in older patients. Few reports describe the clinical outcomes in older patients with idiopathic MN.@*METHODS@#The outcomes of 135 patients with histologically proven MN were analyzed. ‘Older’ was defined as 60 years of age or older at the time of the renal biopsy. The rates of complete remission (CR), progression to end-stage renal disease (ESRD) and infection were compared between older and younger patients.@*RESULTS@#The cumulative event rate for achieving CR was inferior (p = 0.012) and that for requiring renal replacement was higher (p = 0.015) in older patients, and they had a greater risk of infection (p = 0.005). Older age was a significant predictor of a lower rate of CR (adjusted odds ratio [OR], 0.51; 95% confidence interval [CI], 0.26 to 0.98), and was a robust predictor of infection (adjusted OR, 5.27; 95% CI, 1.31 to 21.20). Conservative treatment was associated with a lower remission rate (p = 0.036) and corticosteroid treatment was less effective in achieving CR (p = 0.014), in preventing progression to ESRD (p = 0.013) and in reducing infection (p = 0.033) in older patients. Cyclosporine treatment had similar clinical outcomes with regard to CR, ESRD progression, and infection in older patients.@*CONCLUSIONS@#Older age was independently associated with inferior rates of CR and greater risk of infection. Treatment modalities affected the outcomes of older patients differently in that cyclosporine treatment is predicted to be more useful than corticosteroids.
ABSTRACT
BACKGROUND: Vitamin D is considered to exert a protective effect on various renal diseases but its underlying molecular mechanism remains poorly understood. This study aimed to determine whether paricalcitol attenuates inflammation and apoptosis during lipopolysaccharide (LPS)-induced renal proximal tubular cell injury through the prostaglandin E₂ (PGE₂) receptor EP4. METHODS: Human renal tubular epithelial (HK-2) cells were pretreated with paricalcitol (2 ng/mL) for 1 hour and exposed to LPS (1 μg/mL). The effects of paricalcitol pretreatment in relation to an EP4 blockade using AH-23848 or EP4 small interfering RNA (siRNA) were investigated. RESULTS: The expression of cyclooxygenase-2, PGE₂, and EP4 were significantly increased in LPS-exposed HK-2 cells treated with paricalcitol compared with cells exposed to LPS only. Paricalcitol prevented cell death induced by LPS exposure, and the cotreatment of AH-23848 or EP4 siRNA offset these cell-protective effects. The phosphorylation and nuclear translocation of p65 nuclear factor-kappaB (NF-κB) were decreased and the phosphorylation of Akt was increased in LPS-exposed cells with paricalcitol treatment. AH-23848 or EP4 siRNA inhibited the suppressive effects of paricalcitol on p65 NF-κB nuclear translocation and the activation of Akt. The production of proinflammatory cytokines and the number of terminal deoxynucleotidyl transferase-mediated dUTP nick end labeling-positive cells were attenuated by paricalcitol in LPS exposed HK-2 cells. The cotreatment with an EP4 antagonist abolished these anti-inflammatory and antiapoptotic effects. CONCLUSION: EP4 plays a pivotal role in anti-inflammatory and antiapoptotic effects through Akt and NF-κB signaling after paricalcitol pretreatment in LPS-induced renal proximal tubule cell injury.
Subject(s)
Humans , Apoptosis , Cell Death , Cyclooxygenase 2 , Cytokines , Ergocalciferols , Inflammation , Phosphorylation , Receptors, Prostaglandin E, EP4 Subtype , RNA, Small Interfering , Vitamin DABSTRACT
Occlusive disease of the iliac segment, proximal to the transplant artery (prox-TRAS), in kidney transplant recipients is a rare complication. Prox-TRAS, located in the common iliac artery, is extremely rare in these patients. Herein, we present an interesting case of a common iliac artery stenosis that manifested as decreased allograft function and uncontrolled blood pressure without other typical clinical symptoms. The patient was successfully treated with percutaneous luminal angioplasty and stent insertion.
Subject(s)
Humans , Allografts , Angioplasty , Arteries , Blood Pressure , Constriction, Pathologic , Hypertension , Iliac Artery , Kidney , Kidney Transplantation , Phenobarbital , Stents , Transplant RecipientsABSTRACT
IgG4-related disease (IgG4-RD) is a group of fibro-inflammatory diseases affecting multiple organ systems, pathologically characterized by tissue infiltration with abundant IgG4-positive cells. We here report a case of IgG4-RD that was successfully controlled by mycophenolic acid (MPA). A 62-year-old male visited complaining of dizziness and loss of appetite. He had a clinical history of autoimmune pancreatitis treated with steroids and cyclophosphamide 5 years prior, and had also been diagnosed with Hashimoto's thyroiditis at the age of 55 years. We initially prescribed prednisolone (1 mg/kg), tapered to 5 mg/day over 8 months. However, his eosinophilia gradually deteriorated, and we added cyclophosphamide (1 mg/kg daily) for the next 7 months. His laboratory data fluctuated when he was on low-dose predinisolone and cyclophosphamide, and we thus changed his medication to prednisolone combined with MPA for the following 63 months. Currently, his laboratory findings are stable.
Subject(s)
Humans , Male , Middle Aged , Appetite , Cyclophosphamide , Dizziness , Eosinophilia , Immunoglobulin G , Mycophenolic Acid , Pancreatitis , Prednisolone , Steroids , Thyroid Gland , ThyroiditisABSTRACT
Encapsulating peritoneal sclerosis (EPS) is a rare complication of peritoneal dialysis associated with a high mortality rate. Bacterial peritonitis (BP), a complication of EPS treatment, is uncommon, and treatments for BP are not well known. We report two patients who had undergone steroid treatment who developed BP after hemodialysis transfer. In the first case, we treated the BP with antibiotics and performed several surgical drainage procedures; however, the fluid became too thick to drain. This patient died of malnutrition and sepsis. In the second case, antibiotics and surgical enterolysis with peritonectomy were used to treat the BP. Solid food was accepted on day 7 postoperatively, and the patient was stable after 20 months. Thus, surgical enterolysis with peritonectomy may be a good treatment modality for patients with EPS and BP.
Subject(s)
Humans , Anti-Bacterial Agents , Drainage , Malnutrition , Mortality , Peritoneal Dialysis , Peritoneal Fibrosis , Peritonitis , Renal Dialysis , SepsisABSTRACT
Cryptococcus neoformans is a fungus that causes opportunistic infections in immunocompromised hosts. Skin lesions are found in 10-20% of systemic cryptococcal infections, usually secondary to cryptococcemia, while primary cutaneous cryptococcosis with cryptococcemia is very rare. We report a case of rapidly spreading cryptococcal cellulitis in a 64-year-old male on maintenance hemodialysis taking steroids for encapsulated peritoneal sclerosis. Bluish bullous cellulitis developed on the left forearm and spread rapidly to the other forearm. We identified C. neoformans in the blood and skin lesions. We treated him successfully with liposomal amphotericin B and fluconazole for 15 months. We also review the literature.
Subject(s)
Humans , Male , Middle Aged , Amphotericin B , Cellulitis , Cryptococcosis , Cryptococcus neoformans , Fluconazole , Forearm , Fungi , Immunocompromised Host , Opportunistic Infections , Peritoneal Fibrosis , Renal Dialysis , Skin , SteroidsABSTRACT
BACKGROUND/AIMS: To prevent hypocalcemia after parathyroidectomy (PTX), parenteral calcium is required in addition to oral calcitriol and calcium. After switching to oral calcium, patients can be discharged from the hospital. The aim of this study was to analyze the clinical characteristics and outcomes of PTX performed at a single Korean center and to investigate the associated laboratory factors used to analyze the total amount of postoperative calcium required. METHODS: We enrolled 91 hemodialysis patients undergoing PTX from November 2003 to December 2011. We collected clinical and laboratory data preoperatively, 12 and 48 hours postoperatively, at discharge, and 3 and 6 months postoperatively. RESULTS: In total, 59 patients underwent PTX with autotransplantation (AT), 6 underwent total PTX without AT, 11 underwent subtotal PTX, and 15 underwent limited PTX. Total PTX without AT showed the lowest recurrence rate. At all postoperative time points, the mean levels of serum calcium, phosphorus, and intact parathyroid hormone (iPTH) decreased significantly, compared with preoperative levels; however, alkaline phosphatase (ALP) increased significantly from 48 hours postoperatively to discharge (p < 0.001). On multiple linear regression analysis, the total amount of injected calcium during hospitalization showed a significant correlation with preoperative ALP (p < 0.001), preoperative iPTH (p = 0.037), and Deltaphosphorus at 48 hours (p < 0.001). We developed an equation for estimating the total calcium requirement after PTX. CONCLUSIONS: Preoperative ALP, preoperative iPTH, and Deltaphosphorus at 48 hours may be significant factors in estimating the postoperative calcium requirement. The formula for postoperative calcium requirement after PTX may help to predict the duration of postoperative hospitalization.
Subject(s)
Adult , Aged , Female , Humans , Male , Middle Aged , Young Adult , Administration, Intravenous , Administration, Oral , Biomarkers/blood , Calcium/blood , Calcium Carbonate/administration & dosage , Calcium Compounds/administration & dosage , Calcium Gluconate/administration & dosage , Decision Support Techniques , Dietary Supplements , Hyperparathyroidism, Secondary/blood , Hypocalcemia/diagnosis , Lactates/administration & dosage , Linear Models , Models, Biological , Multivariate Analysis , Parathyroid Hormone/blood , Parathyroidectomy/adverse effects , Phosphorus/blood , Recurrence , Republic of Korea , Retrospective Studies , Risk Factors , Time Factors , Treatment OutcomeABSTRACT
BACKGROUND: Anemia is a major risk factor that contributes to mortality in patients with chronic kidney disease. There is controversy over the optimal hemoglobin (Hb) target in these patients. This study investigated the association between Hb level and mortality in a cohort of hemodialysis (HD) patients in Korea. METHODS: This study was a multicenter prospective observational study of maintenance HD patients that was performed for 5 years in western Seoul, Korea. Three hundred and sixty-two participants were enrolled. Laboratory values and mortality were accessed every 6 months. Repeated measures of laboratory values in each interval were averaged to obtain one semiannual mean value. The Hb values were divided into six groups: (1) Hb or =13 g/dL. We analyzed the odds ratio for all-cause mortality, based on the Hb group, and adjusted for demographics and various laboratory values. Statistics were performed with SAS, version 9.1 software (SAS Institute Inc., Cary, NC, USA). RESULTS: Mortality odds ratios relative to the reference group (10-11 g/dL) in the fully adjusted model were 3.61 for or =13 g/dL (* indicates P<0.05). CONCLUSION: In this study, a Hb level of 10-11 g/dL was associated with the lowest mortality among the groups with Hb level<13 g/dL. Larger interventional trials are warranted to determine the optimal Hb target for Korean HD patients.
Subject(s)
Humans , Anemia , Cohort Studies , Demography , Korea , Mortality , Observational Study , Odds Ratio , Prospective Studies , Renal Dialysis , Renal Insufficiency, Chronic , Risk Factors , SeoulABSTRACT
BACKGROUND/AIMS: Chronic hepatitis B infection is a common cause of secondary membranous nephropathy (MN) in endemic areas. Lamivudine treatment improves renal outcome in patients with hepatitis B virus-associated MN (HBV-MN), but prolonged use leads to the emergence of lamivudine-resistant variants. We describe our experience treating lamivudine-resistant and other strains of HBV-MN with new antiviral drugs. METHODS: Of the 89 patients biopsied and diagnosed with MN from 1996 to 2011, 10 positive for hepatitis B surface antigen were recruited for this study. We investigated the clinical courses, therapeutic responses, and prognoses of patients with HBV-MN. RESULTS: The incidence of HBV-MN among the original 89 patients was 11.2%. Of these patients, four were treated with supportive care and six with antiviral drugs. One of the four patients treated with supportive care had a spontaneous remission. Four of the six patients treated with antiviral drugs were given lamivudine, and the other two were given entecavir. Two of the four patients treated with lamivudine achieved complete remission with seroconversion (i.e., development of anti-hepatitis B e antigen antibodies), whereas the other two had lamivudine-resistant strains, which were detected at 22 and 23 months after lamivudine treatment, respectively. We added adefovir to the treatment regimen for one of these patients, and for the other patient we substituted clevudine for lamivudine. Both of these patients experienced complete remission, as did the two patients initially treated with entecavir, neither of whom showed resistance to the drug. CONCLUSIONS: New nucleoside analogues, such as entecavir, adefovir, and clevudine, can be effective for treatment of HBV-MN, including lamivudine-resistant strains.
Subject(s)
Adult , Female , Humans , Male , Middle Aged , Young Adult , Adenine/analogs & derivatives , Antiviral Agents/therapeutic use , Arabinofuranosyluracil/analogs & derivatives , Drug Resistance, Viral , Glomerulonephritis, Membranous/drug therapy , Guanine/analogs & derivatives , Hepatitis B, Chronic/complications , Lamivudine/therapeutic use , Organophosphonates/therapeutic useABSTRACT
A 27-year-old man presented at the emergency room with hemoptysis. His blood pressure was 180/100 mm Hg, and he had no history of hypertension. Chest radiographs showed bilateral infiltration, suggestive of alveolar hemorrhage. His laboratory data were consistent with acute kidney injury. His serum creatinine level increased abruptly; therefore, renal biopsy was performed. Steroid pulse therapy was administered because of a strong suspicion of immune-mediated pulmonary renal syndrome. Renal biopsy showed proliferative endarteritis, fibrinoid necrosis, and intraluminal thrombi in the vessels without crescent formation or necrotizing lesions. Steroid pulse therapy rapidly tapered and stopped. His serum creatinine level gradually decreased with strict blood pressure control. Ten months after discharge, his blood pressure was approximately 120/80 mm Hg with a serum creatinine level of 1.98 mg/dL. Pulmonary renal syndrome is generally caused by an immune-mediated mechanism. However, malignant hypertension accompanying renal insufficiency and heart dysfunction causing end-organ damage can create a pulmonary hemorrhage, similar to pulmonary renal syndrome caused by an immune-mediated mechanism. The present case shows that hypertension, a common disease, can possibly cause pulmonary renal syndrome, a rare condition.
Subject(s)
Adult , Humans , Male , Glomerulonephritis/diagnosis , Hemoptysis/pathology , Hemorrhage/diagnosis , Hypertension, Malignant/diagnosis , Lung Diseases/diagnosisABSTRACT
Idiopathic thrombocytopenic purpura (ITP) is an autoimmune blood disorder characterized by thrombocytopenia. Common clinical manifestations include bleeding events. Rarely, thrombotic complications may develop in ITP. A 43-year-old man was admitted with dyspnea. His platelet count at admission was 48,000/mm3. The patient had a history of ITP diagnosed 12 years earlier and had been treated with low-dose steroids. Two months before admission, he had been diagnosed with deep vein thrombosis and treated only with clopidogrel due to severe thrombocytopenia. Chest computed tomography showed filling defects in both pulmonary arteries. In the workup for precipitating factors, only lupus anticoagulant was positive. The concomitant administration of warfarin and methylprednisolone was used to treat the pulmonary thromboembolism and ITP, respectively. Six months later, the lupus anticoagulant test remained positive. The patient was confirmed to have a pulmonary thromboembolism due to antiphospholipid syndrome, which might be related to the underlying ITP. After 10 months, his symptoms and radiological findings had improved.
Subject(s)
Adult , Humans , Antiphospholipid Syndrome , Dyspnea , Hemorrhage , Lupus Coagulation Inhibitor , Methylprednisolone , Platelet Count , Precipitating Factors , Pulmonary Artery , Pulmonary Embolism , Purpura, Thrombocytopenic, Idiopathic , Steroids , Thorax , Thrombocytopenia , Ticlopidine , Venous Thrombosis , WarfarinABSTRACT
Angiomyolipoma is a rare, benign, mesenchymal hamartomatous neoplasm consisting of a mixture of adipose tissue, smooth muscle cells, and anomalous blood vessels. The kidney is a common origin site, but extrarenal angiomyolipoma has been reported. Simultaneous involvement of the kidney and regional lymph nodes is uncommon, and may be confused with a metastatic malignant lymph node. Lymph node involvement in angiomyolipoma represents a multifocal invasion of the tumor, rather than metastatic disease. Tumor markers usually associate with malignancy but they have sometimes been detected in benign conditions. We report a case of bilateral renal angiomyolipoma with tuberous sclerosis simultaneously involving regional lymph nodes confused with metastatic malignant lymph node enlargement due to associated elevation of serum tumor markers.