Immunoglobulin G4-Related Kidney Disease with Membranous Proliferative Glomerulonephritis Presenting with a Renal Pelvic Mass / 대한내과학회지

Immunoglobulin G4 (IgG4)-related kidney disease is a chronic immune-mediated fibro-inflammatory disorder characterized by multiple organ infiltration with IgG4-positive plasma cells, storiform fibrosis, and obliterative phlebitis or tumefactive lesions. Previous studies have explored IgG4-related kidney disease, increasing our understanding of its clinical manifestations, and pathological and radiologic findings. However, IgG4-related kidney disease can be misdiagnosed since it mimics malignancies. We report a case of a 77-year-old Korean man diagnosed with IgG4-related kidney disease with membranous proliferative glomerulonephritis, presenting with a renal pelvic mass suspected of being malignant.

Nafcillin-Associated Hypokalemia / 대한내과학회지

Hypokalemia is a common finding in various clinical settings; it is associated with diuretic use and loss of potassium via the gastrointestinal tract. Less common causes are renal tubular acidosis, diabetic ketoacidosis, excess insulin, primary hyperaldosteronism, and medications, such as amphotericin B. Nafcillin, a narrow-spectrum penicillin class antibiotic, which is selectively prescribed for methicillin-susceptible Staphylococcal aureus infections, and is commonly associated with gastrointestinal side effects, such as nausea, vomiting, and abdominal pain. However, in rare cases it can cause hypokalemia, which is usually overlooked. Severe hypokalemia was detected in a 59-year-old male patient hospitalized due to traumatic cerebral hemorrhage who received 12 g of nafcillin per day for more than 2 weeks for sepsis caused by methicillin-sensitive Staphylococcus epidermidis. We confirmed the association between nafcillin and hypokalemia through further evaluation and a review of the relevant literature. Clinicians should be aware of hypokalemia as a complication when using high doses of nafcillin.

Immunoglobulin G4-Related Kidney Disease with Membranous Proliferative Glomerulonephritis Presenting with a Renal Pelvic Mass / 대한내과학회지

Immunoglobulin G4 (IgG4)-related kidney disease is a chronic immune-mediated fibro-inflammatory disorder characterized by multiple organ infiltration with IgG4-positive plasma cells, storiform fibrosis, and obliterative phlebitis or tumefactive lesions. Previous studies have explored IgG4-related kidney disease, increasing our understanding of its clinical manifestations, and pathological and radiologic findings. However, IgG4-related kidney disease can be misdiagnosed since it mimics malignancies. We report a case of a 77-year-old Korean man diagnosed with IgG4-related kidney disease with membranous proliferative glomerulonephritis, presenting with a renal pelvic mass suspected of being malignant.

Nafcillin-Associated Hypokalemia / 대한내과학회지

Hypokalemia is a common finding in various clinical settings; it is associated with diuretic use and loss of potassium via the gastrointestinal tract. Less common causes are renal tubular acidosis, diabetic ketoacidosis, excess insulin, primary hyperaldosteronism, and medications, such as amphotericin B. Nafcillin, a narrow-spectrum penicillin class antibiotic, which is selectively prescribed for methicillin-susceptible Staphylococcal aureus infections, and is commonly associated with gastrointestinal side effects, such as nausea, vomiting, and abdominal pain. However, in rare cases it can cause hypokalemia, which is usually overlooked. Severe hypokalemia was detected in a 59-year-old male patient hospitalized due to traumatic cerebral hemorrhage who received 12 g of nafcillin per day for more than 2 weeks for sepsis caused by methicillin-sensitive Staphylococcus epidermidis. We confirmed the association between nafcillin and hypokalemia through further evaluation and a review of the relevant literature. Clinicians should be aware of hypokalemia as a complication when using high doses of nafcillin.

Acute Kidney Injury after Administering Dapagliflozin to a Diabetic Patient with Acute Cerebral Infarction / 대한내과학회지

Dapagliflozin is a recently developed oral anti-diabetic drug and SGLT2 inhibitor with well-known cardioprotective and renoprotective effects. Although a reduction of the glomerular filtration rate is induced by volume depletion and tubule-glomerular feedback during the early period after administering a SGLT2 inhibitor, the renal prognosis improves more with a decrease of proteinuria. However, the risk of acute kidney injury increases in heart failure and hypovolemia patients, and in those taking certain drugs, such as non-steroidal anti-inflammatory drugs, angiotensin-converting enzyme inhibitor/angiotensin II receptor blockers, or diuretics. We report acute kidney injury after dapagliflozin administration in a diabetic patient with acute cerebral infarction accompanied by right hemiplegia, motor aphasia, and dysphagia.

Acute Kidney Injury after Administering Dapagliflozin to a Diabetic Patient with Acute Cerebral Infarction / 대한내과학회지

Dapagliflozin is a recently developed oral anti-diabetic drug and SGLT2 inhibitor with well-known cardioprotective and renoprotective effects. Although a reduction of the glomerular filtration rate is induced by volume depletion and tubule-glomerular feedback during the early period after administering a SGLT2 inhibitor, the renal prognosis improves more with a decrease of proteinuria. However, the risk of acute kidney injury increases in heart failure and hypovolemia patients, and in those taking certain drugs, such as non-steroidal anti-inflammatory drugs, angiotensin-converting enzyme inhibitor/angiotensin II receptor blockers, or diuretics. We report acute kidney injury after dapagliflozin administration in a diabetic patient with acute cerebral infarction accompanied by right hemiplegia, motor aphasia, and dysphagia.

Cockcroft-Gault, Modification of Diet in Renal Disease, and Chronic Kidney Disease Epidemiology Collaboration equations for estimating glomerular filtration rates in cancer patients receiving cisplatin-based chemotherapy

BACKGROUND: Although the Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI) equation has been recommended for accurate estimates of glomerular filtration rate (eGFR), there is little information regarding differences in GFR estimates obtained using the Cockcroft-Gault (CG) or Modification of Diet in Renal Disease (MDRD) equations in East Asian cancer patients. We investigated discrepancies in GFR and toxicities in patients treated with cisplatin-based chemotherapy using three equations equations. METHODS: A total of 229 patients were retrospectively recruited. We calculated eGFR using the three equations and separated patients into three categories based on GFR 50 (group C) mL/min/1.73m2. We analyzed chemotherapy toxicities. RESULTS: The mean eGFR calculated using the CG was the lowest of the values derived using the three equations. Estimates using the MDRD and CKD-EPI equations resulted in reclassifying 32 (71.1%) and 33 (73.3%) of 45 patients, originally placed in group B using the CG into group C. However, only 1 (7.7%) of 13 patients placed in group B using the MDRD were reclassified into group C using the CKD-EPI. Twenty-eight of 45 patients classified into group B using the CG equation were treated with reduced doses of cisplatin. However, these patients did not show significant differences in toxicities compared with other patients taking full doses of cisplatin. CONCLUSION: The CG equations underestimated GFR compared to the MDRD and CKD-EPI equations. Therefore, when GFR is estimated using CG equations, East Asian cancer patients may receive insufficient doses of chemotherapeutic agents, including cisplatin.

A Case of Mushroom Poisoning with Russula subnigricans: Development of Rhabdomyolysis, Acute Kidney Injury, Cardiogenic Shock, and Death

Mushroom exposures are increasing worldwide. The incidence and fatality of mushroom poisoning are reported to be increasing. Several new syndromes in mushroom poisoning have been described. Rhabdomyolytic mushroom poisoning is one of new syndromes. Russula subnigricans mushroom can cause delayed-onset rhabdomyolysis with acute kidney injury in the severely poisoned patient. There are few reports on the toxicity of R. subnigricans. This report represents the first record of R. subnigricans poisoning with rhabdomyolysis in Korea, describing a 51-year-old man who suffered from rhabdomyolysis, acute kidney injury, severe hypocalcemia, respiratory failure, ventricular tachycardia, cardiogenic shock, and death. Mushroom poisoning should be considered in the evaluation of rhabdomyolysis of unknown cause. Furthermore, R. subnigricans should be considered in the mushroom poisoning with rhabdomyolysis.

A Case of Fulminant Sclerosing Peritonitis Presented Like Acute Culture-Negative Peritonitis and Successfully Treated with Corticosteroid Therapy

Sclerosing peritonitis is an uncommon complication of peritoneal dialysis. It is characterized by peritoneal fibrosis and sclerosis. The most common clinical presentations of sclerosing peritonitis in peritoneal dialysis patients are ultrafiltration failure and small bowel obstruction. The prognosis and response to immunosuppressive therapy of sclerosing peritonitis presenting with ultrafiltration failure or small bowel obstruction are poor. Here, we describe the case of a 28-yr-old man with end-stage renal disease on peritoneal dialysis showing fulminant sclerosing peritonitis presented like acute culture-negative peritonitis and was successfully treated with corticosteroid therapy. It is not well recognized that sclerosing peritonitis may present in this way. The correct diagnosis and corticosteroid therapy may be life-saving in a fulminant form of sclerosing peritonitis.

A Case of Crescentic Glomerulonephritis with Coexisting Anti-Glomerular Basement Membrane Antibodies and Myeloperoxidase-Anti-Neutrophil Cytoplasmic Autoantibodies / 대한내과학회지

Rapidly progressive glomerulonephritis (RPGN) is a clinical syndrome involving abrupt or insidious onset of hematuria, proteinuria, and anemia, and rapidly progressive renal failure. Crescentic glomerulonephritis is a histopathological term for RPGN showing extensive extracapillary proliferation, i.e., crescent formation. There are three major immunopathological categories of crescentic glomerulonephritis: anti-glomerular basement membrane (anti-GBM) antibody disease, immune complex-mediated, and pauci-immune (anti-neutrophil cytoplasmic autoantibody [ANCA]-positive). A small minority of all patients with glomerulonephritis develop crescentic glomerulonephritis. Anti-GBM antibodies and ANCA rarely coexist. There have been a few reports of dual positive crescentic glomerulonephritis with anti-GBM antibodies and ANCA in Korea. Here, we describe the case of a 73-year-old woman showing RPGN clinically and crescentic glomerulonephritis pathologically with coexisting anti-GBM antibodies and myeloperoxidase-ANCA.

Effect of RAAS Inhibition on the Incidence of Cancer and Cancer Mortality in Patients with Glomerulonephritis

Angiotensin II type 1 receptor blocker (ARB), which is frequently prescribed in patients with glomerulonephritis (GN), is suggested to increase the risk of cancer. We registered 3,288 patients with renal biopsy and analyzed the relationship between the use of renin-angiotensin-aldosterone system (RAAS) blockade and the incidence of cancer or cancer mortality. After renal biopsy, cancer developed in 33 patients with an incidence rate of 1.0% (95% of CI for incidence: 0.7%-1.3%). There was no difference in the cancer incidence among the groups according to the use of angiotensin-converting enzyme inhibitors (ACEI) or ARB: 1.2% in the None (23/1960), 0.7% in the ARB-only (5/748), 0.4% in the ACEI-only (1/247), and 1.2% in the ACEI-ARB (4/333) (P = 0.487) groups. The cancer mortality was 2.1%, 0.4%, 0.0%, and 0.3% in None, ACEI-only, ARB-only, and ACEI-ARB group, respectively (P < 0.001). The risk of cancer mortality in patients with ARB was only 0.124 (0.034-0.445) compared to that of non-users of ARB by Cox's hazard proportional analysis. In conclusion, prescription of ACEI or ARB in patients with GN does not increase cancer incidence and recipients of ARB show rather lower rates of all-cause mortality and cancer mortality.

Effect of RAAS Inhibition on the Incidence of Cancer and Cancer Mortality in Patients with Glomerulonephritis

Angiotensin II type 1 receptor blocker (ARB), which is frequently prescribed in patients with glomerulonephritis (GN), is suggested to increase the risk of cancer. We registered 3,288 patients with renal biopsy and analyzed the relationship between the use of renin-angiotensin-aldosterone system (RAAS) blockade and the incidence of cancer or cancer mortality. After renal biopsy, cancer developed in 33 patients with an incidence rate of 1.0% (95% of CI for incidence: 0.7%-1.3%). There was no difference in the cancer incidence among the groups according to the use of angiotensin-converting enzyme inhibitors (ACEI) or ARB: 1.2% in the None (23/1960), 0.7% in the ARB-only (5/748), 0.4% in the ACEI-only (1/247), and 1.2% in the ACEI-ARB (4/333) (P = 0.487) groups. The cancer mortality was 2.1%, 0.4%, 0.0%, and 0.3% in None, ACEI-only, ARB-only, and ACEI-ARB group, respectively (P < 0.001). The risk of cancer mortality in patients with ARB was only 0.124 (0.034-0.445) compared to that of non-users of ARB by Cox's hazard proportional analysis. In conclusion, prescription of ACEI or ARB in patients with GN does not increase cancer incidence and recipients of ARB show rather lower rates of all-cause mortality and cancer mortality.

A Case of Exercise-induced Acute Renal Failure with G774A Mutation in SCL22A12 Causing Renal Hypouricemia

Acute renal failure with severe loin pain which develops after anaerobic exercise is rare. One of predisposing factors of exercise-induced acute renal failure is renal hypouricemia. Idiopathic renal hypouricemia is a genetic disorder characterized by hypouricemia with abnormally high renal tubular uric acid excretion. The mutation in SCL22A12 gene which encodes renal uric acid transporter, URAT1, is the known major cause of this disorder. We here described a 25-yr-old man showing idiopathic renal hypouricemia with G774A mutation in SCL22A12 who presented exercise-induced acute renal failure. There have been a few reports of mutational analysis in Korean idiopathic renal hypouricemia without acute renal failure. This is the first report of genetically diagnosed idiopathic renal hypouricemia with exercise-induced acute renal failure in Korea.

Acute Renal Failure in Paroxysmal Nocturnal Hemoglobinuria / 대한신장학회잡지

It has been reported that paroxysmal nocturnal hemoglobinuria (PNH) kidneys are prone to urinary tract infection, variable functional or anatomical abnormalities, renal venous or intrarenal microvascular thromboembolic events, and variable courses of renal failure. Acute renal failure which is rarely developed in PNH patients is often associated with infection, hemolytic crisis or thrombotic complications. A 40-year-old man, who has been treated as iron deficiency anemia and duodenal ulcer for about 2 months, presented with urinary tract infection, intravascular hemolytic anemia, cholestasis, thrombocytopenia, and acute renal failure. Subsequently he showed progressively aggravating azotemia and refractory hypervolemia during evaluation, and then he eventually was diagnosed as PNH. So, we report a case of PNH which was diagnosed due to the advent of acute renal failure possibly due to hemolytic crisis precipitated by urinary tract infection and documented by flow cytometry, kidney magnetic resonance imaging, and renal biopsy.

Clinical Characteristics of Poor Responders to Erythropoietin among ESRD Patients Undergoing Hemodialysis / 대한신장학회잡지

BACKGROUND: The main pathogenic factor causing anemia in chronic renal failure is the erythropoietin deficiency. However, there are some patients showing poor responses to erythropoietin administration. The purposes of this study were to analyze the clinical parameters of poor responders to erythropoietin among ESRD patients undergoing hemodialysis, and to clarify the major potential factors accounting for poor responses to erythropoietin therapy. METHODS: Eighty-one patients with end-stage renal failure undergoing hemodialysis were included in this study. Poor responders to erythropoietin, defined as patients requiring erythropoietin doses more than 200 U/kg/week subcutaneously to correct anemia, were identified. The hematocrit, erythropoietin dose, erythropoietin resistance index (ERI), and other clinical parameters in 77 patients requiring erythropoietin administration were evaluated and analyzed. RESULTS: Poor responses to erythropoietin were found in 16 patients (20.8%) among 77 patients requiring erythropoietin administration. Serum iron, total iron binding capacity (TIBC), transferrin saturation (TSAT), and albumin concentration were significantly decreased (p or =0.5 mg/dL) was more frequent in poor responders (p<0.01). The independent factor affecting on ERI was TIBC (R=0.44, p<0.01). However, there were no significant difference between poor and good responders in age, gender, duration of dialysis, underlying renal disease, use of ACE inhibitor, dose of dialysis (Kt/V), serum parathyroid hormone, and aluminum concentration. CONCLUSION: TIBC seems to be a predicting factor accounting for poor response to erythropoietin among ESRD patients undergoing hemodialysis.

Serum Magnesium Concentration and Its Clinical Significance in Hemodialysis Patients / 대한신장학회잡지

BACKGROUND: Hypermagnesemia may be implicated to have both harmful and beneficial effects in dialysis patients. It may contribute to osteomalacic renal osteodystrophy and suppression of parathyroid hormone. The purposes of this study were to analyze the serum magnesium concentration in hemodialysis patients, and to clarify the relationship between serum magnesium and intact parathyroid hormone concentration (iPTH) independently of other clinical parameters. METHODS: Eighty-one patients (38 males and 43 females) with end-stage renal failure undergoing maintenance hemodialysis in Dankook University Hospital were included in this study. The mean age was 53+/-12 years and the duration of dialysis was 36+/-35 months. The underlying kidney disease was diabetic nephropathy in 24 patients (30%). The patients used a dialysate magnesium concentration of 1.5 mEq/L. The serum magnesium, iPTH and other clinical parameters were evaluated and analyzed. RESULTS: The mean serum magnesium concentration was 2.78+/-0.45 mEq/L (range 1.50-3.95 mEq/ L). Hypermagnesemia (defined as serum Mg >2.1 mEq/L) was found in 72 patients (89%). The mean iPTH was 128+/-224 pg/mL (range 3-1, 344 pg/mL). The iPTH was significantly low, and the serum aluminum concentration was significantly high in patients (n=28) with more severe hypermagnesemia (defined as serum Mg >3.0 mEq/L). The serum magnesium, aluminum and calcium concentration have significant negative correlations with iPTH respectively (r=-0.27, -0.31, -0.28, p<0.05) in patients (n=55) with relative hypoparathyroidism (defined as iPTH <120 pg/mL). CONCLUSION: Hypermagnesemia was common in hemodialysis patients. High serum aluminum concentration should be considered in patients with moderate to severe hypermagnesemia. Furthermore, hypermagnesemia as well as high serum aluminum and calcium concentration may have a suppressive effect on PTH in patients with relative hypoparathyroidism or adynamic bone disease.

Congenital Nephrogenic Diabetes Insipidus Developing in the Mother and the Son / 대한신장학회잡지

Most cases of congenital nephrogenic diabetes insipidus (NDI) are transmitted in an X-linked recessive manner and are caused by mutations in the vasopressin type 2 receptor (AVPR2) genes on the long arm of the X chromosome (Xq28). In these cases, female carriers are usually asymptomatic, and most patients are male. X-linked NDI is a rare hereditary disease with an estimated prevalence and incidence of approximately four to nine per one million males. Although several cases of congenital NDI diagnosed in the childhood were reported in Korea, there were few reports about congenital NDI diagnosed in the adult and documented by the mutational analysis. We have experienced two cases of congenital NDI developing in the mother and the son, diagnosed in the adult, and confirmed to be caused by mutation (R113W) in AVPR2 gene. Therefore, we report these cases with a brief review of the literature.

Heterozygous Mutations of The Gene for Kir 1.1 (ROMK) in Antenatal Bartter Syndrome Presenting with Transient Hyperkalemia, Evolving to a Benign Course

Bartter-like syndrome encompasses a set of inherited renal tubular disorders associated with hypokalemic metabolic alkalosis, renal salt wasting, hyperreninemic hyperaldosteronism, and normal blood pressure. Antenatal Bartter syndrome, a subtype of Bartter-like syndrome, is characterized by polyhydramnios, premature delivery, life-threatening episodes of fever and dehydration during the early weeks of life, growth retardation, hypercalciuria, and early-onset nephrocalcinosis. Mutations in the bumetanide-sensitive Na-K-2Cl cotransporter (NKCC2) and ATP-sensitive inwardly rectifying potassium channel (ROMK) of the thick ascending limb of Henle's loop have been identified in the antenatal Bartter syndrome. We report the identification of two heterozygous mutations of the gene for Kir 1.1 (ROMK) from an antenatal Bartter syndrome patient who presented at birth with mild salt wasting and a biochemical findings that mimicked primary peudohypoaldosteronism type 1, such as hyperkalemia and hyponatremia, and evolved to a relatively benign course. We have identified amino acid exchanges Arg338Stop and Met357Thr in the gene exon 5 for ROMK by PCR and direct sequencing. Both mutations alter the C-terminus of the ROMK protein, and can affect channel function.

Acute renal failure due to bilateral pyelonephritis / 대한내과학회지

Acute renal failure is a rare complication of acute pyelonephritis. Therefore, acute pyelonephritis is not usually considered in the differential diagnosis of acute renal failure. However, it is important to consider this entity because of potential for recovery of renal function if appropriate early antibiotics are instituted. We report a case of biopsy proven acute pyelonephritis which was manifested as acute renal failure. A 38 year old women was admitted to this hospital owing to abdominal distension and generalized edema. On admission she was started on hemodialysis because of severe hyperkalemia and marked uremic sypmtoms. She had pyuria and hematuria, but no organism was isolated at urine. We initially don't know the cause of renal failure. She was improved with antimicrobial therapy and hemodialysis. A kidney biopsy was performed on the 26th hospital day because of persistent proteinuria. Microscopic examination revealed focal tubular atrophy, necrosis or loss with heavy infilteration of leukocytes and histocytes in interstitium. Atrophic tubules contain pus casts. The patient was treated with ciprofloxacin for 4 weeks. At about 2 month follow up, proteinuria completely disappeared and serum creatinine level decreased to 1.0 mg/dL.

Hemoperfusion and continuous hemodiafiltration in a paraquat poisoned patient / 대한내과학회지

Extracorporeal elimination techniques have been frequently used in paraquat poisoning. But the effect of these techniques is controversial. Plasma paraquat concentration falls rapidly during first few hours after ingestion as the compound is taken by the tissues and excreted by the kidney. Tissue binding is very strong, resulting in slow release of substance from the tissues into the plasma. Thus, once the substance has reached its body stores, it is very difficult to eliminate. For these reasons, any effective extracorporeal elimination therapy should be initiated as early as possible, while paraquat concentrations are high and thereafter, continuous elimination therapy should be maintained to keep plasma paraquat levels as low as possible and remove the toxin as it enters the circulation from tissue stores. We present a case of paraquat poisoning treated by hemoperfusion and hemodiafiltration on this theoretical basis.