Dialysis in renal failure casued by amyloidosis of familial Mediterranean fever. A report of ten cases.

Ten unselected patients with renal failure casued by amyloidosis associated with FMF were treated by regular hemodialysis therapy from 1969 to 1974. They were compared to age-matched control patients treated by hemodialysis in the same unit during the same period, who were suffering from renal failure caused by other disease. Mortality in FMF and control patients was 30% with no significant difference in mean survival, shunt life, serum albumin or hemoglobin levels between the two groups. There was no significant difference in blood pressure measured predialysis or postdialysis in patients with FMF or in controls. The synthetic ACTH stimulation test showed adequate or elevated adrenocortical function. It is concluded that life can be prolonged up to 3 1/2 years by hemodialysis in renal failure caused by amyloidosis complicating FMF, and that renal failure casued by FMF is not a contraindication to regular hemodialysis therapy.

Water immersion in nephrotic syndrome.

Five adults suffering from nephrotic syndrome were immersed up to the neck in 1.3 m of warm water for four hours. There was a mean weight loss of 2 kg; 1 kg was lost through sweat and 1 kg was lost through urine. Thirty-five millimoles of sodium was excreted in the urine in four hours, 15 times more than on control days. The urine became hypotonic to plasma in the first two hours of immersion. Aldosterone levels did not change on immersion in three patients, but fell from elevated levels in two others. It is concluded that water immersion up to the neck in nephrotic patients warrants further investigation as a therapeutic agent in those cases that are not responding to conventional diuretic therapy.

25-Hydroxycholecalciferol levels in bedouins in the Negev.

25-Hydroxycholecalciferol (25-HCC) levels were measured in 31 bedouin females and eight bedouin male tribesmen and compared with the levels in Jewish males and females in Beersheba. In nonpregnant bedouin women the mean 25-HCC level was 25.4 ng/ml +/- 9.78. In pregnant bedouin women the mean was 23.4 ng/ml +/- 8.52. In bedouin males the mean level was 25.7 ng/ml +/- 3.03. In Jewish females, both pregnant and nonpregnant, the levels were higher (32.7 ng/ml +/- 6.02 and 44.3 ng/ml +/- 9.24). Jewish males had levels of 32.8 +/- 6.29 ng/ml. No bedouin had plasma levels below 10 ng/ml, and there was no evidence to suggest deficiency of vitamin D in bedouin males or females.

25 Hydroxycholecalciferol levels in Beduin women in labor and in cord blood of their infants.

25 Hydroxycholecalciferol levels were measured in eight Beduin mothers in labor and in the cord blood of their infants, and compared with values in 42 Sephardi Israeli women in labor and the cord blood of their offspring. Beduin baby cord blood had a mean 25 hydroxycholecalciferol concentration of 3.78 ng/ml +/- 3.22 (SD) being about half of their mothers' level in labor. Values in Sephardi women and cord blood were 3 times higher. PTH levels were lower and calcium concentrations were higher in cord blood (P less than 0.001) in both groups when compared to their mothers' blood. The relevance of these findings to the high incidence of infantile rickets and hyypocalcemia in Beduin children is discussed.

Tuberculosis in patients undergoing maintenance hemodialysis.

Tuberculosis was diagnosed in eight patients undergoing maintenance hemodialysis and occurred with an incidence 10 times that of the general population. Dialysis-associated tuberculosis is characterized by intermittent fever, anorexia, weight loss and hepatomegaly. Ascites was present in 50 per cent of the patients. A recently converted positive tuberculin skin test was observed in five of eight patients. Tuberculosis was extrapulmonary in seven of eight cases and consequently the diagnosis was frequently delayed. Over-all mortality was 37.5 per cent and correlated with the duration of symptoms prior to initiation of therapy. A trial of antituberculous therapy is warranted in patients undergoing dialysis in whom fever of unknown origin, anorexia, weight loss and/or hepatomegaly develop particularly in areas endemic for tuberculosis.

Beta-thromboglobulin levels in the nephrotic syndrome.

Beta-thromboglobulin (BTG), a platelet-specific protein released on platelet aggregation, was measured in 13 patients with clinical and biochemical evidence of the nephrotic syndrome. All 13 patients had increased concentrations of BTG compared to both 10 normal controls and to 12 non-nephrotic azotemic patients (p < 0.001). In five patients with the nephrotic syndrome in remission, the BTG levels returned to normal. These results support the contention that the nephrotic syndrome is associated with a state of hypercoagulability and suggest that increased platelet aggregation may be the primary underlying mechanism.

Prehepatic hyperalimentation.

A technique for prehepatic infusion of parenteral nutrients is described. Portal vein hyperalimentation allows hepatic modification and control of the infused nutrients before delivery of these substances into the general circulation and theoretically should reduce the incidence of metabolic complications of hyperalimentation. The clinical experience with prehepatic infusions is reported and the metabolic investigations are described. Transumbilical catheters provided prehepatic delivery of parenteral nutrients for 1 month after esophagogastrectomy for esophageal malignancy without serious infection or portal vein thrombosis. Close surveillance of blood glucose and serum osmolarity demonstrated metabolic stability during the infusion period. Nitrogen balance studies showed better nitrogen economy than is achieved by infusion of similar solutions into the central systemic circulation. Indirect calorimetry indicated that the nitrogen used for production of energy was less than the amount supplied by prehepatic infusions. The same basic liver function abnormalities encountered with systemic infusion of hyperalimentation solutions were noted. The patients gained weight after esophagogastrectomy and did not experience the attrition from malnutrition which usually occurs in the first several months after esophageal resection.

Silicon metabolism: the basic facts in renal failure.

Silicon levels in plasma and urine have been studied in normal subjects, in chronic renal failure patients, and in regular hemodialysis patients. Plasma levels were elevated in chronic renal failure, and in hemodialysis patients they were three times the control values. Urine silicon excretion is significantly related to creatinine clearance, and to urinary omolality, magnesium and calcium levels. A small amount of residual silicon is found in the hollow fiber artificial kidneys made by some manufacturers. High concentrations of silicon are found in commercial dialysate and pass down a chemical concentration gradient into the blood compartment. It is concluded that silicon is sequestered rapidly in the body during hemodialysis, and that this amounts to 58 g of silicic acid per annum from hemodialysis alone.

An automated technique for the analysis of plasma guanidino acids, and some findings in chronic renal disease.

A sensitive method for the quantitative determination of all guanidino compounds in human serum, including basic compounds such as methylguanidine, was developed by the adaptation of the Technicon automatic amino acid analyzer. Elution buffers with very high pH and molarity are employed for the elution of very basic compounds from the column. Automatic quantitative determination of the plasma guanidino compounds was performed with biacetyl alpha-naphthol, a specific reagent for the guanidino group. This method permitted the quantitative automatic determination of guanidino compounds in less than 1 ml of serum of normal or uremic patients. Guanidino propionic acid and guanidino succinic acid were identified in high concentrations in uremic sera. Methyl guanidine, and guanidine were in smaller concentrations. Guanidino acetic acid and guanidino butyric acid were present in normal plasma in quantities not significantly different from those in uremic plasma.

Aluminum binding activity in canine duodenal mucosal extracts.

Aluminum binding activity in soluble mucosal extracts from canine duodenum could be demonstrated in a single peak corresponding to proteins in the molecular weight range of 9,000 to 10,000 daltons following chromatography on Sephadex G100. Peak calcium binding activity was observed in these same fractions. These results suggest that there exist soluble proteins in duodenal mucosa which have affinity for both aluminum and calcium and that the absorption of aluminum through the gastrointestinal tract may be promoted by these proteins possibly in competition with calcium.

Blood flow rate and access recirculation in hemodialysis.

We studied the effect of extracorporeal blood flow rate (BFR) on access recirculation (recirc) in 19 hemodialysis patients. BUN was determined in simultaneous peripheral (P), arterial (A), and venous (V) blood obtained at BFRs of 200, 400, and 600 ml/min. Percent recirc was calculated for each BFR using the formula (P-A)/(P-V) x 100. Venous drip-chamber (VP) and pre-blood-pump (AP) pressures were measured at each BFR. Fistulograms were performed in 10 patients, and stenoses were identified in 5, all at the proximal (arterial) end of the access. Recirc increased with increasing BFR from 200 to 400 ml/min but increased little from 400 to 600 ml/min. At all BFRs recirc in the stenotic patients was higher than that of non-stenotic or unstudied patients. Urea clearance, corrected for recirc, rose with blood flow both in stenotic and non-stenotic patients. There were no differences in AP or in VP between stenotic and non-stenotic patients. At BFR greater than or equal to 400 ml/min, a recirc threshold of 15% identified stenoses with sensitivity 100% and specificity 71%. We conclude (1) recirc increases with increasing BFR but not enough to outweight the concomitant increase in urea clearance; (2) significant access stenosis and recirc may be present even with low VP; (3) recirc was associated with arterial side stenoses; (4) at BFR greater than or equal to 400 ml/min, access stenosis is associated with recirc greater than 15%.

Effect of nickel on oxygen free radical metabolism. Inhibition of superoxide dismutase and enhancement of hydroxydopamine autoxidation.

The effect of nickel on superoxide dismutase activity (SOD), as well as on rate of hydroxydopamine oxidation, was studied in vitro since lipid peroxidation has been implicated in cell damage by nickel, whose toxicity and carcinogenicity are well established. Nickel strongly inhibits SOD activity. The degree of inhibition is directly proportion to the nickel concentration (tested range 0.066 to 0.33 microgram/mL in the reaction mixture); to the substrate concentration (tested range 0.4 x 10 4M to 1.1 x 10 4M 6-hydroxydopamine); and to reaction mixture. Autoxidation of 6-hydroxydopamine was increased by nickel concentrations higher than 15 micrograms/mL. The combination of excessive oxygen free radical production and inhibition of their elimination by inhibition of SOD activity may contribute to the nickel toxicity that has been reported in industrial accidents, as well as to the high incidence of cancer occurring in nickel workers. It may also contribute to many complications in uremic patients, in whom increased serum nickel levels were reported to be in a similar range to those inhibiting SOD.

Distribution of(31)Silicon-Labeled silicic acid in the rat.

Carrier-free(31)Silicon ((31)Si) prepared by neutron activation, was injected in the form of(31)Si-labeled silicic acid into five albino male rats, and the organ and tissue distribution of labeled silicic acid was determined at sacrifice after 30 min. The kidney was found to contain 0.85% of the injected dose (ID) per gram of tissue; skin had 0.3% ID/G; testes 0.29; bone 0.26; liver 0.22; and brain 0.13. When expressed as % ID/organ, voluntary muscle had 14.6%; skin 10.8; bone 3.4; liver 1.6; kidneys 1.5; testes 0.8, and brain 0.2. These results indicate the need for further research into silicon metabolism in kidney, skin, bone, and brain.

Acid-base parameters in the dehydrated camel.

The effect of prolonged (10 days) dehydration on acid-base parameters of camel blood was examined. The pH and PCO2 levels rose significantly in the course of dehydration. This state was comparable with compensated non-respiratory alkalosis found in other animals. The plasma sodium, and magnesium levels rose significantly also. The plasma oxygen and calcium levels declined significantly. There were no significant changes in potassium and phosphate levels. It is concluded that the changes found in acid-base status following dehydration are further evidence of water preservation mechanisms in the dehydrated camel.