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PubMed1995

Extended measurement of glomerular filtration rate and effective renal plasma flow in ambulatory patients.

We describe a standardized clearance method over 5 h (one hour equilibration followed by eight consecutive 30 min clearance periods [period 2-9]) for the estimation of GFR (iothalamate I125) and ERPF (hippuran I131) during water diuresis in ambulatory and exercising patients. Four groups were examined. In group I (normal controls, n = 15) there were no significant changes in GFR, ERPF and FF (P > 0.10) during repeated clearance periods (mean of period 2-5 versus period 6-9). The reproducibility of the method was studied at a mean interval of 3.7 weeks in a group of patients with stable reduction of GFR (group II, n = 7). The values for GFR, ERPF, FF and RVR did not change significantly in this group, and correlated significantly between repeated studies (r = 0.81 to r = 0.99). In group III (untreated hypertensive patients with reduced GFR, n = 13) there was a time dependent 7.2% decrease in GFR (P < 0.05), significantly different from group I (P < 0.02), a 10.0% decrease in ERPF (P < 0.01) and no significant change in FF (P = 0.08) when the mean of period 2-5 was compared with the mean of period 6-9. In healthy controls (group IV, n = 8) light sustained bicycle exercise (25 W) induced a 7.1% decline in GFR (P < 0.01), 17.4% decline in ERPF (P < 0.001) and a 13.6% increase in FF (P < 0.001). We conclude that ambulatory measurements of GFR and RPF can be carried out over a period of 5 h with satisfactory precision and repeatability. Ambulatory hypertensive patients with moderately reduced GFR showed the same degree of time dependent downward drift of GFR and ERPF without exercise as was seen in healthy individuals during light exercise. Accordingly, in these groups single clearance periods imply a risk for under or overestimation of renal function, and time controls are necessary during clearance studies.

Adult↗
PubMed1995

Biochemical markers of renal disease in primary Sjögren's syndrome.

Primary Sjögren's syndrome (SS) is characterized by an inflammatory process in the salivary and lacrimal glands, but the kidneys may also be involved. Renal tubular functions were studied in 27 patients with SS, all females, age 37-78. Both SS-patients with and without known distal renal tubular acidosis (dRTA) were included, dRTA was found in 18/27 (67%), impaired urine concentrating ability in 13/27 (48%). Hypocitraturia was identified in 20/27 (74%) and reduced tubular reabsorption of phosphate (TRP%) in 18/27 (67%). Tubular proteinuria (alpha 1-mikroglobulin) was present in 11/24 (46%), and tubular enzymuria (NAG) in 7/24 (29%). Hypocitraturia and/or dRTA were found in all patients with any kind of abnormal renal tubular function test. All except one of the patients with dRTA not treated with sodium bicarbonate had hypocitraturia. We conclude that distal tubular dysfunction was common in our SS-patients, but a concommitant proximal dysfunction was also seen. Determination of urinary citrate represents a valuable test for detection of renal disease in SS.

Acetylglucosaminidase↗
PubMed1982

In-vivo and in-vitro hemodialysis studies of thiocyanate.

Dialysis clearance of thiocyanate was studied using in-vivo and in-vitro systems. The in-vivo studies were performed in a patient with renal failure receiving sodium nitroprusside infusion for accelerated hypertension. In-vitro studies were carried out under experimental conditions similar to those of the in-vivo experiment. Plasma thiocyanate level consistently fell with single passage through the dialyzer. In-vivo dialysance of thiocyanate averaged 82.8 ml/min as compared to urea dialysance of 129.6 ml/min. The in-vitro studies revealed an average thiocyanate dialysance of 102.3 as compared to a urea dialysance of 138.6 ml/min. Removal of thiocyanate by hemodialysis was further verified by recovery of significant amounts of thiocyanate in the outgoing dialysate. The thiocyanate clearance calculated directly from the amount recovered in the dialysate and mean plasma concentration was 82.2 ml/min, a value closely approximating that obtained using the transdialyzer concentration gradient. We conclude that hemodialysis is effective in removing thiocyanate and can be used as adjunct in the treatment of thiocyanate toxicity particularly in the presence of renal failure in which thiocyanate excretion is impaired.

Adult↗
PubMed2006

Two cases of isolated diffuse mesangial sclerosis with WT1 mutations.

Here we report two cases of isolated diffuse mesangial sclerosis (IDMS) with early onset end-stage renal failure. These female patients did not show abnormalities of the gonads or external genitalia. Direct sequencing of WT1 PCR products from genomic DNA identified WT1 mutations in exons 8 (366 Arg>His) and 9 (396 Asp>Tyr). These mutations have been reported previously in association with Denys-Drash syndrome (DDS) with early onset renal failure. Therefore we suggest that, at least in part, IDMS is a variant of DDS and that investigations for the WT1 mutations should be performed in IDMS patients. In cases with identified WT1 mutations, the same attention to tumor development should be required as in DDS patients, and karyotyping and serial abdominal ultrasonograms to evaluate the gonads and kidney are warranted.

Base Sequence↗