A Rare Case of Pulmonary Arteriovenous Malformation Caused by Hereditary Hemorrhagic Telangiectasia in a Hemodialysis Patient / 대한내과학회지

Hereditary hemorrhagic telangiectasia (HHT) is an uncommon autosomal dominant disorder resulting in vascular malformation, such as pulmonary arteriovenous malformation (PAVM). Here, we report a rare case of pulmonary arteriovenous malformation caused by HHT in a hemodialysis (HD) patient. A 34-year-old man receiving maintenance HD via radiocephalic arteriovenous fistula developed progressive dyspnea without definite pulmonary edema. His mother had been diagnosed with HHT. He had experienced multiple episodes of epistaxis and had been intermittently treated with blood transfusions because of severe anemia. Blood gas analysis showed hypoxia. Chest computed tomography revealed multiple dilated vessels of variable sizes, continuous with the pulmonary artery throughout both lung fields, consistent with PAVM. After treating pulmonary artery embolization at the largest PAVM, he recovered from his dyspnea symptoms and hypoxia.

Mortality prediction of serum neutrophil gelatinase-associated lipocalin in patients requiring continuous renal replacement therapy

Background/Aims@#We investigated whether serum neutrophil gelatinase-associated lipocalin (NGAL) can predict mortality in patients with acute kidney injury (AKI) requiring continuous renal replacement therapy (CRRT). @*Methods@#This study enrolled 169 patients who underwent serum NGAL testing at CRRT initiation from June 2017 to January 2019. The predictive power of serum NGAL level for 28-day mortality was compared to the Acute Physiology and Chronic Health Evaluation-II (APACHE-II) score and Sequential Organ Failure Assessment (SOFA) score via area under the receiver operating characteristic curve (AuROC) value. @*Results@#There were 55 survivors and 114 non-survivors at 28 days post-CRRT initiation. Median serum NGAL level was significantly higher in the non-survivor group than in the survivor group (743.0 ng/mL vs. 504.0 ng/mL, p = 0.003). The AuROC value of serum NGAL level was 0.640, which was lower than APACHEII score and SOFA score values (0.767 and 0.715, respectively). However, in the low APACHE-II score group (< 27.5), AuROC value of serum NGAL was significantly increased (0.698), and it was an independent risk factor for 28 day-mortality (hazard ratio, 2.405; 95% confidence interval, 1.209 to 4.783; p = 0.012). @*Conclusions@#In patients with AKI requiring CRRT, serum NGAL levels may be useful for predicting short-term mortality in those with low APACHE-II scores.

A Rare Case of Pulmonary Arteriovenous Malformation Caused by Hereditary Hemorrhagic Telangiectasia in a Hemodialysis Patient / 대한내과학회지

Hereditary hemorrhagic telangiectasia (HHT) is an uncommon autosomal dominant disorder resulting in vascular malformation, such as pulmonary arteriovenous malformation (PAVM). Here, we report a rare case of pulmonary arteriovenous malformation caused by HHT in a hemodialysis (HD) patient. A 34-year-old man receiving maintenance HD via radiocephalic arteriovenous fistula developed progressive dyspnea without definite pulmonary edema. His mother had been diagnosed with HHT. He had experienced multiple episodes of epistaxis and had been intermittently treated with blood transfusions because of severe anemia. Blood gas analysis showed hypoxia. Chest computed tomography revealed multiple dilated vessels of variable sizes, continuous with the pulmonary artery throughout both lung fields, consistent with PAVM. After treating pulmonary artery embolization at the largest PAVM, he recovered from his dyspnea symptoms and hypoxia.

Recurrent Severe Hyponatremia in a Patient with Sjögren’s Syndrome

Sjögren's syndrome (SS) is an autoimmune disease that presents with exocrine gland dysfunction. Renal involvement is common in SS and often results in tubulointerstitial nephritis, renal tubular acidosis, and Fanconi’s syndrome. Electrolyte imbalances are commonly the first symptom of renal involvement of SS. The most common feature of dysnatremia in SS is hypernatremia with diabetes insipidus. However, cases of hyponatremia with syndrome of inappropriate antidiuretic hormone secretion (SIADH) are rarely reported in patients with SS. Herein, we report a case of recurrent severe SIADH in a patient with SS.

Clinical outcomes of infection-related hospitalization in incident peritoneal dialysis patients

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.

Recurrent Severe Hyponatremia in a Patient with Sjögren’s Syndrome

Sjögren's syndrome (SS) is an autoimmune disease that presents with exocrine gland dysfunction. Renal involvement is common in SS and often results in tubulointerstitial nephritis, renal tubular acidosis, and Fanconi’s syndrome. Electrolyte imbalances are commonly the first symptom of renal involvement of SS. The most common feature of dysnatremia in SS is hypernatremia with diabetes insipidus. However, cases of hyponatremia with syndrome of inappropriate antidiuretic hormone secretion (SIADH) are rarely reported in patients with SS. Herein, we report a case of recurrent severe SIADH in a patient with SS.

Clinical outcomes of infection-related hospitalization in incident peritoneal dialysis patients

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.

Successful transcatheter closure of an inferior sinus venosus atrial septal defect

No abstract available.

Tumor Lysis Syndrome in a Patient with Metastatic Colon Cancer after Treatment with 5-Fluorouracil/Leucovorin and Oxaliplatin: Case Report and Literature Review / Journal of the Korean Cancer Association, 대한암학회지

Development of tumor lysis syndrome (TLS) may occur after chemotherapy or spontaneously in bulky or rapidly growing tumors. This syndrome is frequent but preventable in patients with hematologic malignancies. TLS following therapy has been reported infrequently in various types of solid tumors. TLS associated with oxaliplatin containing chemotherapy in a solid tumor has never been reported. A 59-year-old man received 5-fluorouracil, leucovorin, and oxaliplatin (FOLFOX) chemotherapy for metastatic colon cancer. Development of TLS occurred three days after administration of chemotherapy. Two days later, his abnormal laboratory findings were recovered with appropriate management. To the best of our knowledge, the current case is the first report on development of acute TLS following oxaliplatin containing chemotherapy in a patient with colon cancer. We also review the literature on tumor lysis syndrome in patients with colorectal cancer.

A Case of Asymptomatic Severe Pulmonary Valve Stenosis with Patent Foramen Ovale and Patent Ductus Arteriosus in an Adult / 대한내과학회지

Pulmonary valve stenosis (PS) is the 3rd most common form of adult congenital heart disease. The patient was a 56-year-old woman, with known congenital heart disease but who was not receiving any treatment as she was not functionally limited. A two-dimensional echocardiogram showed severe right ventricular hypertrophy, pulmonary valve thickening and systolic doming. A color Doppler revealed a retrograde flow from the aorta to the left pulmonary artery. She had severe heart failure on the right hand side from a PS but did not display any symptoms. We hypothesized that she had a retrograde flow to the pulmonary circulation through a patent ductus arteriosus (PDA), through which oxygenated blood could be supplied to the systemic circulation and, hence, no hypoxia. We attempted a balloon valvuloplasty for the PS followed by a device closure for the PDA. Here we report on this adult female with severe PS but lacking any symptoms, due to the presence of a PDA.

A Case of Asymptomatic Severe Pulmonary Valve Stenosis with Patent Foramen Ovale and Patent Ductus Arteriosus in an Adult / 대한내과학회지

Pulmonary valve stenosis (PS) is the 3rd most common form of adult congenital heart disease. The patient was a 56-year-old woman, with known congenital heart disease but who was not receiving any treatment as she was not functionally limited. A two-dimensional echocardiogram showed severe right ventricular hypertrophy, pulmonary valve thickening and systolic doming. A color Doppler revealed a retrograde flow from the aorta to the left pulmonary artery. She had severe heart failure on the right hand side from a PS but did not display any symptoms. We hypothesized that she had a retrograde flow to the pulmonary circulation through a patent ductus arteriosus (PDA), through which oxygenated blood could be supplied to the systemic circulation and, hence, no hypoxia. We attempted a balloon valvuloplasty for the PS followed by a device closure for the PDA. Here we report on this adult female with severe PS but lacking any symptoms, due to the presence of a PDA.