Sclerosing encapsulating peritonitis: a case successfully treated with immunosuppression
Department of Nephrology, Hospital de Santo Antonio, Porto, Portugal.
Perit Dial Int. 1999; 19(5): 478-81
PMID: 11379862 [PubMed - indexed for MEDLINE]
Ineffectiveness of dialysis in transthyretin (TTR) clearance in familial amyloid polyneuropathy type I, in spite of lower stability of the TTR Met30 variant
Department of Nephrology, Hospital Geral de Santo Antonio, Instituto Nacional de Saude Dr. Ricardo Jorge, Porto, Portugal.
Clin Nephrol. 1999; 51(1): 45-9
BACKGROUND: Familial Amyloid Polyneuropathy (FAP) is an hereditary form of systemic amyloidosis related to a mutant transthyretin (TTR). The renal disease ranges from proteinuria to end-stage renal failure (ESRF), with replacement of renal function by dialysis. In comparison with FAP patients with normal renal function, the progression of the neurologic disease seems to be retarded in FAP patients on dialysis.
PATIENTS AND METHODS: We evaluated the influence of hemodialysis and hemodiafiltration on plasma TTR levels in 6 FAP patients with ESRF, which were on regular hemodialysis for 4 months to 6 years, prior to this study. Hemodialysis was performed over a two-week period, one week with a cellulose triacetate membrane and the other with a polysulfone membrane. In the third week, patients were submitted to hemodiafiltration. Plasma TTR levels were measured at the beginning, 60 min, 120 min, and at the end of each session. We also evaluated the TTR adsorbed by the membrane and in the dialysate.
RESULTS: TTR levels did not change significantly with the dialysis. The total amount of TTR adsorbed to the membrane was always < 2 mg and found in the dialysate < 1 mg. Hemodialysis and hemodiafiltration were ineffective in removing TTR, in spite of lower stability of the TTR Met30 variant.
CONCLUSION: The protective feature of hemodialysis on the progression of the amyloidosis is not due to the clearance of this abnormal protein from plasma.
PMID: 9988146 [PubMed - indexed for MEDLINE]
Primary antiphospholipid syndrome presented by total infarction of right kidney with nephrotic syndrome
Nephrology Department, Hospitais da Universidade de Coimbra, Portugal.
Clin Nephrol. 1999; 52(1): 56-60
We report the case of a young woman with primary antiphospholipid syndrome (APS), which presented with acute renal failure, hypoproteinemia, hypoalbuminemia and nephrotic proteinuria. Investigations showed total infarction of right kidney by extensive arterial and vein thrombosis and presence of anticardiolipin antibodies IgG isotype (anti-beta2-glycoprotein I-positive). She was submitted to right nefrectomy and initiated anticoagulant therapy. After nefrectomy, the postoperative period was marked by the development of arterial hypertension and persistence of nephrotic syndrome. Hypertension was treated with antihypertensive drugs (IECA, beta-blocker and calcium antagonist). As the nephrotic syndrome persisted despite anticoagulant and antihypertensive therapy, the patient was treated with oral corticosteroids. Her renal function improved, hypoproteinemia and hypoalbuminemia corrected to normal values and proteinuria decreased to subnephrotic value. We discuss the unusual presentation of this case of primary antiphospholipid syndrome with total unilateral renal thrombosis and nephrotic syndrome that respond to anticoagulant, antihypertensive and corticosteroid therapy.
PMID: 10442498 [PubMed - indexed for MEDLINE]