Necrotizing sarcoid granulomatosis with uveitis: a variant of sarcoidosis?

A patient with malaise, uveitis and a nodular infiltrate in the left lower lobe of the lung is described. An open lung biopsy established the diagnosis of necrotizing sarcoid granulomatosis. The differential diagnosis of necrotizing sarcoid granulomatosis with sarcoidosis and angiocentric granulomatosis (Wegener's disease) is extensively discussed. Our case illustrates that NSG and sarcoidosis could be pathogenetically related.

Primary systemic amyloidosis complicated by massive thrombosis.

We present a case of primary systemic amyloidosis complicated by multiple thrombotic events and initially presenting with a massive thrombosis of the inferior vena cava. Widespread infiltration of the vascular tree by amyloid was found at the time of autopsy. In addition, we report successful treatment of the massive inferior vena cava thrombosis with systemic thrombolysis.

Peritoneal dialysis in acute renal failure due to cholesterol embolization: two cases of recovery of renal function and extended survival.

Recovery of renal function and extended survival after acute renal failure due to cholesterol embolization are possible, as demonstrated by the case history of two patients. Both were treated with peritoneal dialysis during their acute insult and had a serum creatinine of 1.7 mg/100 ml and 2.4 mg/100 ml, respectively, 12 and 60 months after diagnosis. Peritoneal dialysis is the preferred procedure for the replacement of renal function in these patients since it achieves good control of blood pressure and volume overload and avoids the need for vascular access and heparin administration.

Guanidino compounds in serum and cerebrospinal fluid of non-dialyzed patients with renal insufficiency.

Twelve guanidino compounds were determined in simultaneously sampled serum and cerebrospinal fluid of eight non-dialyzed patients with renal insufficiency. Liquid cation exchange chromatography with a highly sensitive fluorescence detection method was used. In patients with serum urea levels about 10 times higher than in controls, the levels of guanidinosuccinic acid, creatinine, guanidine and methylguanidine, in serum as well as in cerebrospinal fluid, are at least 10 times higher than in control subjects. The levels of argininic acid and N-alpha-acetylarginine (in serum) and gamma-guanidinobutyric acid (in cerebrospinal fluid) are slightly increased (less than 10 X). The levels of the other guanidino compounds are close to normal values. A significant positive correlation exists between the guanidinosuccinic acid, creatinine and guanidine levels in serum and cerebrospinal fluid. The accumulation of several experimentally proven toxic guanidino compounds could contribute to the complex nervous system symptomatology and the hematological complications seen in renal insufficiency.

6-keto-PGF1 alpha levels and prostacyclin therapy in 2 adult patients with hemolytic-uremic syndrome.

Evidence supports the hypothesis that plasma prostacyclin activity is deficient in hemolytic-uremic syndrome (HUS). We studied 2 adult patients with HUS. Plasma levels of 6-keto-PGF1 alpha, the stable metabolite of prostacyclin, were measured by radioimmunoassay. Both patients were found to have elevated 6-keto-PGF1 alpha levels. These findings are in contradiction with the prostacyclin deficiency hypothesis and with earlier reports of low or undetectable plasma levels of this metabolite. The patients were treated with IV prostacyclin after a single plasma exchange. The first patient, admitted with advanced renal failure, obtained a rapid remission but renal function did not recover; the second patient, admitted with a less pronounced degree of renal failure, reacted slowly to therapy but renal function partially recovered. We believe that, if any benefit is to be expected from prostacyclin therapy in HUS, it should be started early in the course of the disease.