Renal biopsy in elderly patients: clinicopathological correlation in 117 Korean patients.

AIM: In the elderly with renal disease, the clinical presentations are frequently inconsistent with the pathologic findings. We tried to clarify the differences in pathological findings between the young and the elderly, in Korea and in Western countries, and the usefulness of a percutaneous renal biopsy in the elderly with renal disease. PATIENTS AND METHODS: We analyzed the clinical presentations and spectrums of renal histopathology by reviewing medical records and renal biopsy reports retrospectively in 117 Korean patients aged 60 years or more with renal disease. RESULTS: 85 patients had primary renal disease. The remaining 32 patients had renal diseases associated with systemic conditions. Out of the 85 patients with primary renal disease, 61 cases presented as idiopathic nephrotic syndrome. Compared with renal biopsy results of younger adult patients (age 15-59, n = 1,908), membranous nephropathy, crescentic glomerulonephritis, membranoproliferative glomerulonephritis, amyloidosis, light chain disease, and thrombotic thrombocytopenic purpura were more prevalent, but IgA nephropathy and lupus nephritis were less common in the elderly patients. In clinical presentation, nephrotic syndrome and rapidly progressive renal failure were more prevalent, but asymptomatic urinary abnormality was less common in elderly patients. The responsiveness to treatment was good in elderly patients with minimal-change lesion (complete remission in all patients) but poor in crescentic glomerulonephritis, IgA nephropathy, and membranoproliferative glomerulonephritis. From the above findings, the clinical presentation, patterns of histopathology and responsiveness to treatment of elderly Korean patients were similar to those of the younger Korean control group and the Western elderly group. CONCLUSION: Percutaneous renal biopsy is a useful diagnostic aid and can be used as a therapeutic guideline even in elderly patients with renal disease.

ACE gene polymorphism and renal responsiveness to ACE inhibitors in IgA nephropathy patients.

We examined the renal responsiveness to ACE inhibitor in IgA nephropathy (IgAN) patients according to the grouping of ACE gene polymorphism. Sixty one patients diagnosed as IgAN by renal biopsy and prescribed with ACE inhibitors were enrolled. Genomic DNA was extracted from whole blood and PCR was performed. The I/D polymorphism was determined by the presence of the 287 bp fragment in intron 16 of chromosome 17. During the follow-up period (mean; 44.6 months, median: 44.5 months, 5 to 113 months), the blood pressure of 61 patients was controlled below 130/80 mmHg. The renal responsiveness was determined by the degree of changes of proteinuria at 12 months after initiation of ACE inhibitors and by the slope of reciprocal variation of the serum creatinine against follow-up duration ¿(1/Cr2-1/Cr1)/durations¿. The distribution of the II, ID and DD genotype among 61 patients was 21, 16 and 24 patients, respectively. There were no differences among three genotypes in age, sex, the number of patients with initial blood pressure over 140/90 mmHg, initial serum creatinine level, the number of patients with initial azotemia (> 1.4 mg/dL) and with initial 24-hr proteinuria amount over 2.0 g. Significant anti-proteinuric effect of ACE inhibitor was found in IgAN (p = 0.001), but no significant difference was found among genotypes. Significant difference (p = 0.011) was noticed between II type and DD type in the slope of reciprocal variation of the serum creatinine against follow-up duration. In conclusion, efficacy of ACE inhibitors on renal function preservation in IgAN was more pronounced in DD genotype than II genotype.

Nonocclusive mesenteric ischemia in a patient on maintenance hemodialysis.

UNLABELLED: Nonocclusive mesenteric ischemia (NOMI) is known to occupy about 25% to 60% of intestinal infarction. NOMI has been reported to be responsible for 9% of the deaths in the dialysis population and the postulated causes of NOMI include intradialytic hypotension, atherosclerosis and medications, such as diuretics, digitalis and vasopressors. Clinical manifestations, such as fever, diarrhea and leukocytosis, are nonspecific, which makes early diagnosis of NOMI very difficult. CASE: A 66-year-old woman on maintenance hemodialysis for 5 years was admitted with syncope, abdominal pain and chilly sensation. Since 7 days prior to admission, blood pressure on the supine position during hemodialysis had frequently fallen to 80/50 mmHg. Four days later, she complained of progressive abdominal pain. Rebound tenderness and leukocytosis (WBC 13900/mm3) with left shift were noted. Stool examination was positive for occult blood. Abdominal CT scan showed a distended gall bladder with sludge. Under the impression of acalculous cholecystitis, she was operated on. Surgical and pathologic findings of colon colon were compatible with NOMI. Because of recurrent intradialytic hypotension, we started midodrine 2.5 mg just before hemodialysis and increased the dose up to 7.5 mg. After midodrine therapy, blood pressure during dialysis became stable and the symptoms associated with hypotension did not recur. CONCLUSION: As NOMI may occur within several hours or days after an intradialytic hypotensive episode, abdominal pain should be carefully observed and NOMI should be considered as a differential diagnosis. In addition, we suggest that midodrine be considered to prevent intradialytic hypotensive episodes.

Homocyst(e)ine and atherosclerosis in patients on chronic hemodialysis.

Hyperhomocyst(e)inemia is an established risk factor for atherosclerosis. We performed this study to identify the correlating variables and risk factors for atherosclerosis, as measured by the atherosclerotic score (AS), and to determine the relative risk for cardiovascular disease in relation to plasma homocyst(e)ine levels in patients on chronic hemodialysis. We evaluated and measured 61 patients on chronic hemodialysis for clinical and biochemical parameters including atherosclerotic score (AS) and plasma homocyst(e)ine. We divided patients into high and low groups, first, by the mean AS, and second, by the median value of plasma total homocyst(e)ine levels. Then we compared the variables between the two groups. Out of the 61 patients, the median plasma total homocyst(e)ine level was 24.4 micromol/L (mean+/-SD, 27.7+/-17.4; range, 9.8-127.4 micromol/L), and the median AS was 5 (mean+/-SD, 6.2+/-2.8; range, 3-13) out of a possible 20 points. AS was significantly correlated with plasma total homocyst(e)ine levels (r=0.37) and age (r=0.67). Through multivariate analysis, plasma total homocyst(e)ine level and age were determined as significant risk factors for the high-AS group (p<0.05). However, plasma total homocyst(e)ine level did not correlate with age (p>0.05). Eighteen of the 61 patients, presented with cardiovascular disease until the present study, had an AS>6. Cardiovascular disease was found more often in the high-homocyst(e)ine group (>24.4 micromol/L) than in the low-homocyst(e)ine group (odds ratio, 9.3; 95% confidence interval, 2.3-37.4). Regardless of age, hyperhomocyst(e)inemia (especially homocyst(e)ine levels >24.4 micromol/L) is a risk factor that can be modified for the development of cardiovascular disease in patients on chronic hemodialysis.

A case of Wegener's granulomatosis complicated by diffuse pulmonary hemorrhage and thrombotic thrombocytopenic purpura.

Wegener's granulomatosis is a distinct form of necrotizing granulomatous vasculitis which usually affects the kidneys and the upper and lower respiratory tracts. Unusual manifestations have also been reported, and these include colitis, urethritis and diabetes insipidus. We describe a case of Wegener's granulomatosis which presented with rapidly progressive renal insufficiency, sudden deafness, red eye, facial palsy, and complicated by uncommon manifestations that were diffuse pulmonary hemorrhage and thrombotic thrombocytopenic purpura.

Sjögren's syndrome with acute renal failure.

We experienced a 65-year-old woman with Sjögren's syndrome who presented with acute renal failure, hypergammaglobulinemia with monoclonal gammopathy, and hypocomplementemia. She improved with steroid pulse therapy (methylprednisolone 0.5 g/day for 3 days). This patient had also sensorineural hearing loss, symmetric sensory polyneuropathy of legs, and interstitial lung disease. Ten months after recovery from acute renal failure, low-dose oral prednisolone (0.1 mg/kg/day) was withdrawn. On the third month of steroid withdrawal, acute renal failure recurred with hypergammaglobulinemia, hyperamylasemia, and autoimmune cholangitis-like biochemical derangements, which also responded to steroid pulse therapy (methylprednisolone 0.3 g/day for 3 days). When we would withdraw steroid in a patient with visceral involvement of Sjögren's syndrome, we should consider multiple clinical and laboratorial variables, including erythrocyte sedimentation rate, serum levels of IgG, total protein, C3/C4, CRP, amylase, lipase, and alkaline phosphatase. We report this case which exhibited various unusual manifestations with a review of literature.

Delayed onset of diuresis in a patient with acute renal failure due to hemorrhagic fever with renal syndrome who also developed anterior hypopituitarism.

A 23-year-old man developed acute renal failure (ARF) due to hemorrhagic fever with renal syndrome (HFRS). The patient also developed anterior hypopituitarism as a complication of HFRS. The patient's oliguric phase was very much prolonged for over 10 days before the diuresis began. The urine output during the oliguric phase was near anuric (< 50 ml/day). Interestingly, the patient began to diurese just after the institution of glucocorticoid and thyroid hormone replacement therapy. The plasma atrial natriuretic polypeptide went up to a smaller peak (150.0 pg/ml) at the onset of diuresis compared with 15 other patients (292.4 +/- 190.4 pg/ml) who did not develop anterior hypopituitarism. The delayed onset of diuresis and smaller increase of plasma ANP may have a causal relationship with the patient's hypopituitarism.

Arginine vasopressin gene expression in rats with puromycin-induced nephrotic syndrome.

Nephrotic syndrome is characterized by water and sodium retention, which leads to edema formation. The nonosmotic stimulation of arginine vasopressin (AVP) release from the pituitary gland has been implicated to be one of the important factors of abnormal water retention in patients with nephrotic syndrome. It is not known, however, whether nephrotic syndrome is associated with stimulation of hypothalamic vasopressin gene expression. Puromycin aminonucleoside is known to cause altered glomerular permeability, which results in experimental nephrotic syndrome in rats. In the present study, therefore, AVP gene expression has been studied in the hypothalamus of rats with puromycin aminonucleoside-induced nephrotic syndrome (PNS). Nephrotic syndrome was induced by a single intravenous injection of puromycin aminonucleoside (50 mg/kg body weight). Nephrotic syndrome was confirmed by urinary protein excretion (control 20.8 +/- 3.5 mg/24 hr v PNS 273.9 +/- 41.4 mg/24 hr; P < 0.0001, n = 6) and serum albumin concentrations (control 4.52 +/- 0.07 g/dL v PNS 2.96 +/- 0.22 g/dL; P < 0.001, n = 6). In PNS rats, plasma AVP was significantly higher than in control rats (control 0.77 +/- 0.10 pg/mL v PNS 2.13 +/- 0.42 pg/mL; P < 0.005, n = 12), even though there were no differences in plasma osmolality (control 292.0 +/- 2.0 mOsm/kg H2O v PNS 290.3 +/- 2.5 mOsm/kg H2O; P = NS, n = 12) or serum sodium concentration (control 142.7 +/- 0.7 v PNS 142.1 +/- 1.1; PNS, n = 12).(ABSTRACT TRUNCATED AT 250 WORDS)

A case of primary hyperparathyroidism with hypercalcemic nephropathy in children.

Primary hyperparathyroidism is a rare disease in children and is characterized by conspicuous skeletal and renal changes. A 12 year old male patient presented with symptoms of polydipsia, polyuria, general weakness, nausea, and vomiting which had begun 3 months earlier, and showed typical laboratory findings of primary hyperparathyroidism. Confirmatory diagnosis was made by elevated parathyroid hormone concentration in serum, technetium-thallium subtraction scan imaging method and histopathologic finding of chief cell hyperplasia. The laboratory findings revealed elevated levels of BUN, creatinine and decreased GFR. Kidney biopsy showed typical calcium deposits in tubules with marked tubulointerstitial infiltration. After subtotal parathyroidectomy, clinical findings improved remarkably.

A case of cerebral infarction associated with positive antiphospholipid antibody in a systemic lupus patient.

Antiphospholipid antibody syndrome is a newly-defined clinical entity of arterial thrombosis, venous thrombotic events, recurrent spontaneous abortion and thrombocytopenia in the presence of antiphospholipid antibodies. We have experienced a 23-year-old male SLE patient with positive anticardiolipin antibody who presented with left hemiparesis and paresthesia. The clinical and laboratory findings were compatible with the criteria for SLE and he was found to have anticardiolipin antibody, thrombocytopenia, prolonged partial thromboplastin time and cerebral thrombosis. Initially, he was treated with high dose steroid and warfarin and now he is being followed up with warfarin and steroid.

An outbreak of Heinz body positive hemolytic anemia in chronic hemodialysis patients.

During the four month period, from December 1988 to March 1989, there was an outbreak of Heinz body positive hemolytic anemia in 34 patients undergoing hemodialysis in a 500-bed hospital, Seoul, Korea. The episodes of hemolysis were not reduced by changing the charcoal column and reverse osmosis system, or by adding ascorbic acid to the dialysate. The concentrations of nitrate, copper, aluminum and zinc in the treated water were all within the standards for hemodialysis. The chloramine concentration of the treated water was over 0.6 mg/L, markedly exceeding the allowable level of 0.1 mg/L. This high level of chloramine was proved to be due to the contamination of the water source by raw sewage. After we changed the source of water supply to another, no more episodes of hemolytic anemia occurred. It is concluded that chloramine is one of the major contaminants causing dialysis-induced hemolytic anemia and regular determinations are necessary, especially during winter and dry seasons.

Mycobacterium fortuitum peritonitis associated with continuous ambulatory peritoneal dialysis.

Runyon group IV atypical mycobacteria, Mycobacterium fortuitum, is an environmental organism and is capable of producing a variety of clinical infections such as cutaneous infection, abscess and pulmonary and ocular infection. Rarely, it has been a documented cause of peritonitis in patients receiving continuous ambulatory peritoneal dialysis (CAPD). We report a case of M. fortuitum peritonitis in a patient undergoing CAPD and emphasize the importance of mycobacterial cultures in patients with CAPD-associated peritonitis whose routine culturing yields no organisms repeatedly.

A clinical study of hemorrhagic fever with renal syndrome caused by Seoul virus infection.

The clinical findings of 29 patients with hemorrhagic fever with renal syndrome (HFRS) caused by Seoul virus were evaluated and compared with the previously reported clinical findings of classic Korean hemorrhagic fever (KHF). The diagnoses of these patients were made by hemagglutination inhibition test. The results were as follows: 1) The disease occurred predominantly in males with a high incidence in the third and fourth decades of life. 2) The highest incidence of the disease occurred in October-December. 3) Major symptoms were fever, abdominal or flank pain, vomiting and myalgia. 4) Major signs were petechia, CVA tenderness, pharyngeal injection, and conjunctival infection, but these signs were much less common than in patients with classic KHF. 5) The treatments were mainly conservative and there was no fatal case in the study subjects. These findings suggest that the clinical course of Seoul virus infection may be much milder than that of classic KHF and the outcome may be more favorable.

Acute renal failure associated with hematuria in IgA nephropathy.

Severe acute deterioration of renal function, which occurred during the episode of massive gross hematuria, is described in two patients with IgA nephropathy (IgAN). The renal failure abated gradually after dialytic support. Renal pathology in each case revealed focal proliferative glomerulonephritis with less than 14% of the glomeruli affected by crescents or sclerosis. In contrast to the mild glomerular lesions, acute tubular cell injury in relation to phagocytosis of erythrocytes or pigments and/or tubular obstruction was prominent. We think our observations support the previous reports that tubular lesions in relation to heavy glomerular bleeding may be a cause of severe acute renal failure in patients with IgAN.