Hepatorenal syndrome: Current concepts and future perspectives

Hepatorenal syndrome (HRS), a progressive but potentially reversible deterioration of kidney function, remains a major complication in patients with advanced cirrhosis, often leading to death before liver transplantation (LT). Recent updates in the pathophysiology, definition, and classification of HRS have led to a complete revision of the nomenclature and diagnostic criteria for HRS type 1, which was renamed HRS-acute kidney injury (AKI). HRS is characterized by severe impairment of kidney function due to increased splanchnic blood flow, activation of several vasoconstriction factors, severe vasoconstriction of the renal arteries in the absence of kidney histologic abnormalities, nitric oxide dysfunction, and systemic inflammation. Diagnosis of HRS remains a challenge because of the lack of specific diagnostic biomarkers that accurately distinguishes structural from functional AKI, and mainly involves the differential diagnosis from other forms of AKI, particularly acute tubular necrosis. The optimal treatment of HRS is LT. While awaiting LT, treatment options include vasoconstrictor drugs to counteract splanchnic arterial vasodilation and plasma volume expansion by intravenous albumin infusion. In patients with HRS unresponsive to pharmacological treatment and with conventional indications for kidney replacement therapy (KRT), such as volume overload, uremia, or electrolyte imbalances, KRT may be applied as a bridging therapy to transplantation. Other interventions, such as transjugular intrahepatic portosystemic shunt, and artificial liver support systems have a very limited role in improving outcomes in HRS. Although recently developed novel therapies have potential to improve outcomes of patients with HRS, further studies are warranted to validate the efficacy of these novel agents.

Renal Infarction in Vascular Ehlers-Danlos Syndrome / 대한내과학회지

Renal infarction is a condition caused by sudden disturbance of renal arterial blood flow, which occurs frequently in cardioembolic disease, renal artery injury (trauma, Marfan syndrome, rarely Ehlers-Danlos syndrome), and in association with a hypercoagulable state. Ehlers-Danlos syndrome is a rare hereditary connective tissue disease characterized by skin fragility, hyperelasticity, hypermobility of small joints, and easy bruising. Among the subtypes, vascular Ehlers-Danlos syndrome is a very rare genetic disease caused by a mutation in the COL3A1 gene. As blood vessels are fragile due dysfunctional collagen synthesis, complications of bleeding due to arterial dissection or rupture are common in patients with this disease. Here, we report a case of vascular Ehlers-Danlos syndrome diagnosed based on a renal infarction caused by thrombus and renal artery injury; we also present a review of the relevant literature.

Minimal Change in Nephrotic Syndrome Associated with Mixed Connective Tissue Disease / 대한내과학회지

Mixed connective tissue disease (MCTD) is a rheumatologic disorder that has combined features of systemic lupus erythematosus, systemic sclerosis, and rheumatoid arthritis with the anti-U1 ribonucleoprotein (RNP) antibody. Although renal involvement is not included in any of the diagnostic criteria, it has been observed in 20% of MCTD patients. The most common renal disease is immune complex nephropathy, histologically classified as membranous glomerulonephritis, but minimal change disease (MCD) is very rare and has only been reported once previously. A previously healthy 33-year-old woman presented with generalized edema, puffy hands, arthralgia, and Raynaud’s phenomenon. We diagnosed MCD with proteinuria (> 9 g/day) and hypoalbuminemia (< 1.0 g/dL) by renal biopsy, and MCTD with a simultaneously high titer of the anti-U1 RNP antibody. The patient was treated with 200 mg/day cyclosporine and 30 mg/day prednisolone. The proteinuria and hypoalbuminemia normalized 2 months later. We report a patient with severe MCD associated with MCTD.

A Case of Sweet’s Syndrome with Acute Kidney Injury in a Patient with Myelodysplastic Syndrome / 대한내과학회지

Sweet’s syndrome (SS) is an autoimmune-mediated acute febrile neutrophilic dermatosis with a number of possible etiologies, including infection, malignancy, and drug reactions. In contrast to its original description, it can rarely involve extracutaneous organs, including the central nervous system, cardiovascular system, lung, liver, gastrointestinal tract, spleen, and bone. To our knowledge, there have been only three cases of SS accompanied by acute kidney injury worldwide, and this is the first report in which the patient recovered completely from acute kidney injury as well as cutaneous lesions with early steroid administration. Here, we report a case of SS with acute kidney injury in a patient with myelodysplastic syndrome (MDS) whose skin lesions and renal function recovered fully with early diagnosis and steroid therapy along with a review of the relevant literature.

Minimal Change in Nephrotic Syndrome Associated with Mixed Connective Tissue Disease / 대한내과학회지

Mixed connective tissue disease (MCTD) is a rheumatologic disorder that has combined features of systemic lupus erythematosus, systemic sclerosis, and rheumatoid arthritis with the anti-U1 ribonucleoprotein (RNP) antibody. Although renal involvement is not included in any of the diagnostic criteria, it has been observed in 20% of MCTD patients. The most common renal disease is immune complex nephropathy, histologically classified as membranous glomerulonephritis, but minimal change disease (MCD) is very rare and has only been reported once previously. A previously healthy 33-year-old woman presented with generalized edema, puffy hands, arthralgia, and Raynaud’s phenomenon. We diagnosed MCD with proteinuria (> 9 g/day) and hypoalbuminemia (< 1.0 g/dL) by renal biopsy, and MCTD with a simultaneously high titer of the anti-U1 RNP antibody. The patient was treated with 200 mg/day cyclosporine and 30 mg/day prednisolone. The proteinuria and hypoalbuminemia normalized 2 months later. We report a patient with severe MCD associated with MCTD.

Numerical expression of volume status using the bioimpedance ratio in continuous ambulatory peritoneal dialysis patients: A pilot study

BACKGROUND: Volume overload results in higher mortality rates in patients on continuous ambulatory peritoneal dialysis (CAPD). The ratio of bioimpedance (RBI) might be a helpful parameter in adjusting dry body weight in CAPD patients. This study examined whether it is possible to distinguish between non-hypervolemic status and hypervolemic status in CAPD patients by using only RBI. METHODS: RBI was calculated as follows: RBI = impedance at 50 kHz/impedance at 500 kHz. Based on the experts’ judgements, a total of 64 CAPD patients were divided into two groups, a non-hypervolemic group and a hypervolemic group. The RBI was measured from right wrist to right ankle (rw-raRBI) by bioimpedance spectroscopy (BCM®, Fresenius Medical Care) before and after the peritosol was emptied. Other RBIs were measured from the right side of the anterior superior iliac spine to the ipsilateral ankle (rasis-raRBI) to control for the electro-physiological effects of peritoneal dialysate. RESULTS: The mean rw-raRBI of non-hypervolemic patients was higher than that of hypervolemic patients in the presence (1.141 ± 0.022 vs. 1.121 ± 0.021, P < 0.001) of a peritosol. Likewise, the mean rasis-raRBI of non-hypervolemic patients was higher than that of hypervolemic patients (presence of peritosol: 1.136 ± 0.026 vs. 1.109 ± 0.022, P < 0.001; absence of peritosol: 1.131 ± 0.022 vs. 1.107 ± 0.022, P < 0.001). CONCLUSION: The volume status of CAPD patients was able to be simply expressed by RBI. Therefore, this study suggests that when patients cannot be analyzed using BCM, RBI could be an alternative.

Clinical Outcomes of Cryopreserved Arterial Allograft Used as a Vascular Conduit for Hemodialysis

This single center cohort study aimed to test the hypothesis that use of a cryopreserved arterial allograft could avoid the maturation or healing process of a new vascular access and to evaluate the patency of this technique compared with that of vascular access using a prosthetic graft. Between April 2012 and March 2013, 20 patients underwent an upper arm vascular access using a cryopreserved arterial allograft for failed or failing vascular accesses and 53 using a prosthetic graft were included in this study. The mean duration of catheter dependence, calculated as the time interval from upper arm access placement to removal of the tunneled central catheter after successful cannulation of the access, was significantly longer for accesses using a prosthetic graft than a cryopreserved arterial allograft (34.4 ± 11.39 days vs. 4.9 ± 8.5 days, P < 0.001). In the allograft group, use of vascular access started within 7 days in 16 patients (80%), as soon as from the day of surgery in 10 patients. Primary (unassisted; P = 0.314) and cumulative (assisted; P = 0.673) access survivals were similar in the two groups. There were no postoperative complications related to the use of a cryopreserved iliac arterial allograft except for one patient who experienced wound hematoma. In conclusion, upper arm vascular access using a cryopreserved arterial allograft may permit immediate hemodialysis without the maturation or healing process, resulting in access survival comparable to that of an access using a prosthetic graft.

Clinical Outcomes of Cryopreserved Arterial Allograft Used as a Vascular Conduit for Hemodialysis

This single center cohort study aimed to test the hypothesis that use of a cryopreserved arterial allograft could avoid the maturation or healing process of a new vascular access and to evaluate the patency of this technique compared with that of vascular access using a prosthetic graft. Between April 2012 and March 2013, 20 patients underwent an upper arm vascular access using a cryopreserved arterial allograft for failed or failing vascular accesses and 53 using a prosthetic graft were included in this study. The mean duration of catheter dependence, calculated as the time interval from upper arm access placement to removal of the tunneled central catheter after successful cannulation of the access, was significantly longer for accesses using a prosthetic graft than a cryopreserved arterial allograft (34.4 ± 11.39 days vs. 4.9 ± 8.5 days, P < 0.001). In the allograft group, use of vascular access started within 7 days in 16 patients (80%), as soon as from the day of surgery in 10 patients. Primary (unassisted; P = 0.314) and cumulative (assisted; P = 0.673) access survivals were similar in the two groups. There were no postoperative complications related to the use of a cryopreserved iliac arterial allograft except for one patient who experienced wound hematoma. In conclusion, upper arm vascular access using a cryopreserved arterial allograft may permit immediate hemodialysis without the maturation or healing process, resulting in access survival comparable to that of an access using a prosthetic graft.

Acute Kidney Injury in Critically Ill Patients / 대한내과학회지

Despite substantial advances in dialysis techniques and machines, acute kidney injury (AKI) requiring renal replacement therapy (RRT) is still associated with up to 60% in-hospital mortality. However, there is little information on whether RRT overcomes the significant morbidity and mortality of AKI. What is most important in the treatment of AKI is that RRT is not a cause-specific therapy but life-supportive management. This review discusses the indications of, proper initiation of, and optimal prescription for RRT to improve the survival of critically ill patients with AKI.

Renal Replacement Therapy in Acute Kidney Injury: Indication, Proper Initiation, and Prescription / 대한내과학회지

Despite substantial advances in dialytic techniques and machines, acute kidney injury (AKI) requiring renal replacement therapy (RRT) is associated with up to 60% in-hospital mortality. But, there is no full detail of definite RRT to overcome the significant morbidity and mortality of AKI. What is most important in the treatment for AKI is that RRT is not a cause-specific therapy but a life-supportive management. This review discusses the indications of, proper initiation of, and optimal prescription for RRT to improve the survival of the patients with AKI.

Cryoglobulinemic vasculitis and monoclonal gammopathy in end-stage renal disease

No abstract available.

Comparison of Drug-Eluting Stent and Bare Metal Stent in Reducing Adverse Cardiac Event after Coronary Stenting in Hemodialysis Patients / 대한신장학회지

PURPOSE: The patients treated with hemodialysis have been known to have a high 1-year mortality rate after percutaneous coronary intervention (PCI). We evaluated the clinical benefits of drug-eluting stent (DES), compared to bare-metal stent (BMS) in Korean hemodialysis patients. METHODS: We studied 72 hemodialysis patients (M:F=49:23, age 60+/-11 years) treated with percutaneous coronary intervention (PCI) from January, 1999 to February, 2006. Forty four patients (M:F=28: 16, age 60+/-11 years) treated with DES and 28 patients (M:F=21:7, age 60+/-12 years) with BMS were enrolled. A composite of major adverse cardiac and cerebral endpoints (MACCE) was defined as all-cause death, myocardial infarction, stroke, and target-vessel revascularization (TVR). We compared the incidence of MACCE between DES and BMS group. RESULTS: A composite of MACCE occurred in none in DES group (n=44) and 4 in BMS group (n=28) within 3 months after coronary stenting (Kaplan-Meier survival analysis: 0% vs 14%, respectively, p= 0.02). After 3 months, there was no difference in the incidence of primary end points between two groups. Multi-vessel disease (OR 2.47, 95% CI 1.02-6.11, p<0.05) was associated with a significant increase in the incidence of MACCE within 18 months after coronary artery stenting CONCLUSION: DES may be superior to BMS in reducing adverse cardiac outcome at early period after coronary stenting in HD patients. However, this study showed no significant difference with the lapse of time.

Immunoglobulin G4 Related Retroperitoneal Fibrosis with Acute Kidney Injury: Report of A Case / 대한신장학회지

Immunoglobuin G4 (IgG4)-related sclerosing disease is a recently recognized syndrome characterized clinically by male predominance, elevated serum IgG4 level, and good response to steroid therapy. The pathologic characteristics are extensive lymphoplasmacytic infiltration with IgG4-positive cell and irregular fibrosis in various organs (pancreas, bile duct, salivary gland, retroperitoneum, kidney and lung). Some inflammatory pseudotumors may be involved in this disease. Herein, we experienced a case of IgG4-related retroperitoneal fibrosis with postrenal acute kidney injury and inflammatory pseudotumor of thyroid in a 31-year-old female who underwent insertion of double J catheter at both ureters and received corticosteroid treatment.

A Case of Familial Atypical Hemolytic Uremic Syndrome Associated with Complement Factor H Mutation in Adults / 대한신장학회지

The hemolytic uremic syndrome is a clinical syndrome defined by the presence of thrombocytopenia, microangiopathic hemolytic anemia and acute renal failure. Atypical hemolytic uremic syndrome (aHUS) which is not usually associated with prodromal symptoms, especially diarrhea, has a higher mortality rate and a stronger tendency to progress to chronic renal failure. In approximately 30-50% of patients with aHUS, mutations have been detected in complement factor H, membrane cofactor protein or factor I. Mutations in the complement regulator factor H are the most frequent and have a very poor prognosis, with most patients developing ESRD. We have experienced a 33-year-old man with a family history of renal failure diagnosed as aHUS resulted from factor H mutation, for whom we carried out hemodialysis, plasmapheresis and other conservative management.

Comparison of Erythropoietic Effect between Epoetin-alpha and Darbepoetin-alpha in Hemodialysis Patients: A Randomized Crossover Study / 대한신장학회지

PURPOSE: We aim to compare the erythropoietic effects of epoetin-alpha (EA, 4000 IU SC thrice a week) with those of darbepoetin-alpha (DA, 60ug IV weekly, conversion rate to EA=200:1). METHODS: Forty one stable hemodialysis patients were enrolled in this randomized crossover study. After a washout period of erythropoietin stimulating agents (ESA), the patients with hemoglobin (Hb) level of 11.0 g/dL, we stopped ESA. When Hb level decreased to 30% change in EA efficiency relative to DA efficiency. CONCLUSION: There was no significant difference in erythropoietic parameters for both EA and DA.

SIADH and Guillain-Barre Syndrome Associated with Pulmonary Tuberculosis / 대한신장학회지

Hyponatremia is one of the most common electrolyte disturbances in nephrologic clinical setting. SIADH is one of the causes of hyponatremia and can be accompanied with various conditions such as malignancies, infections and nervous system diseases. Guillain-Barre syndrome is an acute inflammatory polyneuropathy. It is reported that SIADH can be accompanied with Guillain-Barre syndrome although the mechanism is unclear. Symptoms of Guillain- Barre syndrome such as general weakness, decreased consciousness, and seizure are similar to those of hyponatremia. Thus the diagnosis of Guillain-Barre syndrome can be delayed if they coexist. Because Guillain-Barre syndrome leads to severe respiratory failure in its disease course and mechanical ventilatory assistance is mandatory for such cases, early diagnosis and treatment is critical. Korea is still endemic area for tuberculosis and clinicians often experience pulmonary tuberculosis in practice. But pulmonary tuberculosis-associated SIADH and Guillain-Barre syndrome were not reported in Korea. So far, 2 cases of gastrointestinal infection-associated SIADH and Guillain-Barre syndrome were reported. Hence, authors report here the case of SIADH associated Guillain-Barre syndrome associated with pulmonary tuberculosis.

A Case of Interstitial Pneumonitis Associated with Sirolimus Treatment in Renal Transplantation Recipient / 대한신장학회지

Sirolimus is a promising immunosuppressive drug for renal transplantation to avoid nephrotoxicity of calcineurin inhibitor. However, it has been associated with uncommon but, important pulmonary toxicity. We present a case of sirolimus related dyspnea with abnormal chest radiographic finding in a 63 year old male renal transplantation recipient. There was no evidence of bacterial, fungal, or viral infection. Dose reduction of sirolimus resulted in a significant improvement of the symptoms and chest radiographic finding.

Calcium-Sensing Receptor Gene Polymorphism is Associated with the Parathyroid Response to Low Calcium Dialysate in Hemodialysis Patients with Low Parathyroid Hormone Secretion / 대한신장학회지

PURPOSE: Low level of parathyroid hormone (PTH) is a risk factor that might cause hip fracture in dialysis patients. Low calcium dialysate (LCD) has been suggested as an approach to increase PTH level. The calcium-sensing receptor (CaSR) polymorphism is known to be associated with the sensitivity to extracellular calcium. METHODS: We prospectively investigated the role of genetic polymorphism of CaSR codon 990 as one cause of the different parathyroid responses to LCD in maintenance hemodialysis (HD) patients. 48 patients, using 3.5 mEq/L calcium dialysate, with intact PTH below 100 pg/ml for the last one year underwent HD sessions on 2.5 mEq/L calcium dialysate for 12 weeks. Serum intact PTH, total calcium, phosphorus, alkaline phosphatase (ALP) and bone-specific ALP (BAP) were measured monthly. The CaSR gene from peripheral lymphocytes was amplified to confirm the genotype by polymerase chain reaction. RESULTS: According to the CaSR genetic polymorphism, subjects were divided into 3 groups, A/A (14.6 %), A/G (45.8%) and G/G (39.6%). Twelve weeks later, intact PTH (48.5+/-25.4 to 89.0+/-49.4 pg/mL, p<0.01), and ALP (78.7+/-25.7 to 87.4+/-27.2 IU/L, p<0.01) increased significantly in G/G group, but not in non-G/G groups. However, BAP significantly increased in both G/G group (24.3+/-11.9 to 29.5+/-10.6 U/L, p<0.01) and in non-G/G groups (21.4+/-4.5 to 26.1+/-9.1 U/L, p<0.01). During the study period, levels of corrected total calcium and phosphorus were not significantly changed. CONCLUSION: The CaSR polymorphism, G/G genotype, strongly influenced the responsiveness of parathyroid gland to LCD, compared with non-G/G genotypes. However, bone formation may occur actively on LCD, irrespective of CaSR genetic polymorphism.

Comparison of Serum Beta 2-Microglobulin and 24 hour Urinary Creatinine Clearance as a Prognostic Factor in Multiple Myeloma

A new staging system for multiple myeloma (MM) has utilized serum concentrations of beta 2-microglobulin (S beta2 M) and albumin as important prognostic factors for survival. Since S beta2 M is an indicator of glomerular filtration rate, we compared the prognostic values of S beta2 M and 24-hr urinary creatinine clearance (Ccr) in patients with MM. We retrospectively reviewed the records of 170 MM patients from January 1996 to November 2003 whose 24-hr urinary Ccr was available at the time of diagnosis. We found that pretreatment S beta2 M was inversely related to Ccr (Spearman's correlation coefficient=-0.787). In univariate analysis, the hazard ratio (HR) of death was 1.043 (p<0.001) for S beta2 M and 0.985 (p<0.001) for Ccr. Multivariate analysis showed that S beta2 M (HR 1.030, p=0.010) and Ccr (HR 0.993, p=0.059) were significant prognostic factors in patients' survival. In conclusion, 24-hr urinary Ccr may be utilized for staging of patients with MM.

Distribution of Cardiac Troponin T in Continuous Hemodialysis Patients and Clinical Characteristics of Patients with Elevated Cardiac Troponin T / 대한신장학회잡지

PURPOSE: The leading cause of death of end-stage renal failure is cardiovascular disease. Elevated cardiac troponin T (cTnT) is associated with the high incidence and prevalence of cardiovascular disease and increased mortality and morbidity. Therefore the aim of this study was to examine the distribution of cTnT in continuous hemodialysis patients and clinical significance of elevated cTnT. METHODS: We studied 183 asymptomatic patients who were undergoing chronic continuous hemodialysis treatment. Predialysis blood samples also used to measure hematocrit, albumin, total cholesterol. We evaluated the history of coronary artery disease and the etiologies of renal disease using chart reviews and patients interviews and cardiac status were determined by electrocardiography, two-dimensional echocardiography and coronary angiography. Predialysis cTnT was measured by a second-generation assay, Elecsys 1010 and we considered patients with serum cTnT>0.1 ng/mL as positive. RESULTS: Forty two patients (23%) had cTnT greater than 0.1 ng/mL. Ischemic heart disease was observed in 18.6% (34/183), diabetes mellitus (DM) in 53.6% (98/183), hypertension in 92.3% (169/183) and left ventricular hypertrophy in 67.2% (123/183). cTnT revealed significantly higher level in the patients with age over 60, male, DM, pulse rate over 60 mmHg, ischemic heart disease (IHD), hematocrit under 36% and albumin under 3.7 g/dL. cTnT levels significantly correlated to ischemic heart disease, DM and male. CONCLUSION: In hemodialysis patients, cTnT level is related to age, sex, DM, pulse rate, IHD, hematocrit and albumin. A prospective study is necessary to provide information on the effect of correcting the controllable factors on cTnT level and cardiovascular mortality in maintenance hemodialysis patients.