Proximal tubules of the kidney produce liver fatty acid-binding protein (L-FABP) and the level of messenger RNA is increased by physical and chemical stimuli. In this study, changes in urinary excretion of L-FABP were examined in 18 patients without renal dysfunction and who had undergone angiography using iodinated contrast media. On the day following angiography, the urinary excretion of L-FABP was markedly increased by 578%(p<0.001) as compared with the value before the administration of contrast media. The urinary N-acetyl-β-D-glucosaminidase excretion was also increased by +93%, however, the extent was less marked than for L-FABP. The urinary L-FABP excretion swiftly returned to the basal level on the second day after the angiography. No patient showed an increase in serum creatinine concentration, and the changes in urinary excretions of other microproteins, such as β2-microglobulin and α1-microglobulin, were not significant. These results suggest that the urinary excretion of L-FABP sensitively reflects the injurious stress on renal tubules. Monitoring of urinary L-FABP may be useful in detecting the development of contrast media nephropathy at an early stage.
Antiphospholipid antibody syndrome (APS) is characterized by the presence of repeated arterial and venous thrombosis, recurrent fetal loss and thrombocytopenia. Recently, renal involvement associated with APS is being increasingly recognized and discussed. In most cases, there has been a vascular nephropathy characterized by small vessel vaso-occulusive lesions associated with fibrous intimal hyperplasia of the interlobular arteries, thrombosis and focal cortical atrophy. We report a case of a 38-year-old patient with primary APS. Renal biopsies were performed three times in 26 years. Various glomerular and vascular lesions associated with APS were observed and discussed.
A Sixty-eight-year-old man complained of shortness of breath on exercise since the spring of 2001. An abnormal lung shadow was pointed out on chest X-ray and progression of renal dysfunction and high γ globulinemia were detected out on blood examination. He was admitted to the Department of Respiratory Disease in our hospital in March 2003, because of bilateral lower lung interstitial shadow, severe anemia (Hb 7.9g/dl), and renal dysfunction (S-Cr 1.9mg/dl). He was found to have hypergammaglobulinemia (IgG 2, 997mg/dl), positive RO/SS-A antigen, high serum KL-6 level (2, 050U/ml), and increased urinary excretion of β2-microglobulin (β2MG). Both Gum test and Schirmer test results were positive. Lip biopsy showed cell infiltration to the salivary glands and he was diagnosed as having Sjögren's syndrome. Renal biopsy showed diffuse interstitial cell infiltration and a Trans Bronchoscopic Lung Biopsy (TBLB) showed fibrotic thickness and lymphocyte infiltration in the alveolar septum. Accordingly, he was diagnosed as having Sjögren's syndrome complicated with both interstitial nephritis and interstitial pneumonitis. He was treated by high-dose corticosteroid therapy and anticoagulant heparin. His laboratory data showed that both serum KL-6 and urinary β2MG were reduced. Chest CT showed remarkable improvement of the interstitial shadow. A second renal biopsy performed at ten weeks after the beginning of treatment showed remarkable improvement of the interstitial cell infiltration. This is a rare case of Sjögren's syndrome complicated with interstitial nephritis and interstitial pneumonitis, treated successfully with high dose corticosteroid therapy. Both complications showed immediate improvement with high-dose corticosteroid therapy, suggesting that early steroid therapy is effective for both complications.
We report a 71-year-old woman with autosomal dominant polycystic kidney disease (ADPKD), who presented with hepatic encephalopathy. She was diagnosed as having ADPKD at 61 years of age. Thereafter, her renal function gradually worsened and she was admitted to our hospital because of encephalopathy and end-stage renal failure. The main laboratory findings were as follows: BUN 77mg/dl; creatinine 9.0mg/dl; ammonia 573μg/dl. Hepatic encephalopathy was improved after hemodialysis and administration of lactulose. The liver demonstrated multiple cysts on computed tomography. Angiography demonstrated that the peripheral branch of the portal vein was stenotic and a spleno-renal shunt was detected. We considered that portal hypertension was caused by multiple liver cysts, and that hepatic encephalopathy was caused by the spleno-renal shunt. It is generally considered that severe hepatic complications are rare in ADPKD, but this case suggested the need to screen for the development of hepatic lesions in ADPKD.
A 31-year-old man was admitted to the hospital because of a low-grade fever, general malaise, nausea, vomiting, and a poor appetite. On admission his renal function was severely deteriorated (serum creatinine 16.12mg/dl, BUN 163mg/dl), and he had severe anemia (Hb 7.5g/dl) and thrombocytopenia (67, 000/μl). A radiological examination revealed the presence of multiple cysts in his kidneys bilaterally. The patient was diagnosed as having end-stage renal disease due to polycystic kidney disease, and hemodialysis was started on the day of admission. After the initiation of hemodialysis, his symptoms and laboratory tests improved, except for anemia and thrombocytopenia. He was noted to have marked splenomegaly and dilation of the portal vein, raising the suspicion of portal hypertension as the cause of the splenomegaly and pancytopenia. To treat his pancytopenia (anemia and thrombocytopenia) and to determine the reason for his portal hypertension, a splenectomy and openwedge biopsy of the liver were performed. Histological findings in the liver included extensive fibrosis of the portal areas with an excess of moderately dilated bile ducts, compatible with a diagnosis of congenital hepatic fibrosis. After splenectomy, his red blood cell and platelet counts returned to normal, and he was discharged on maintenance dialysis. Congenital hepatic fibrosis is often associated with autosomal recessive polycystic kidney disease (ADPKD), but not with autosomal dominant polycystic kidney disease (ADPKD). However, both his mother and older brother had multiple renal cysts, indicating that this was an unusual case of ADPKD complicated by congenital hepatic fibrosis.
We report a case of thrombotic thrombocytopenic purpura (TTP) with a positive Coombs' test. A 59-year-old female was admitted to our hospital in February, 1997 with symptoms of heart failure. Ultrasound cardiography showed moderate pericardiac effusion and she was diagnosed as having pericarditis. After admission she had anorexia and her urine volume was reduced. Laboratory tests showed anemia and thrombocytopenia. Her Coombs' test result was positive. Her renal function gradually worsened and her conscious level was reduced. We diagnosed her as TTP and judged that she needed hemodialysis. We performed plasma exchange and started steroid therapy. The renal biopsy was compatible with TTP. After treatment, her level of consciousness improved, but her renal function did not improve. On the 51st hospital day she fell into acute respiratory distress syndrome (ARDS) and entered ICU. We considered ARDS caused by infection and continued treatment, but she died of shock and lactate acidosis. Activity of von Willebrand factor-cleaving protease in our case was 15% before the first PE, and 25% just before death. A case of TTP without collagen disease usually shows a negative Coombs' test result. We think that this was a rare case in which autoimmune hemolytic anemia was supervened with TTP.
A 48-year-old male was referred to our university hospital for severe azotemia with muscle cramp. He had been taking Chinese herbs as a traditional medicine to reduce hyperuricemia for about 9 months. Urinalysis showed trace proteinuria and hematuria without any casts. Renal glucosuria was also observed. In addition to azotemia, hyperchloremic metabolic acidosis and severe anemia were revealed. Hemodialysis was conducted and his general condition improved. A renal biopsy specimen revealed severe interstitial fibrosis and tubular atrophy with cellular degeneration. No remarkable glomerular changes were observed except for wrinkling of the basement membrane in a few glomeruli. Aristolochic acid was detected in the Chinese herbs, leading to the diagnosis of aristolochic acid nephropathy (AAN). His renal dysfunction was considered to be irreversible and he underwent maintenance hemodialysis. In Japan, AAN or Chinese herbs nephropathy decreased after an outbreak from 1995 to 2000. The public should be warned again that Chinese herbs, which are not permitted by the Japanese government, may contain aristolochic acid.