Evaluation of Plasma Beta-2-microglobulin in Patients with the Nephrotic Syndrome.

In patients with the nephrotic syndrome, it is often desirable to assess the disease process, not only by proteinuria but also by indices of glomerular inflammatory process. We investigated the importance of beta-microglobulin (betaM) as a means of assessing renal function in patients with the nephrotic syndrome with normal or abnormal values of creatinine clearance. There were 46 patients (mean age, 42.2 + 10.4 years; male/female (M/F) ratio = 31/15) and 35 healthy controls (mean age 39 + 4.5 years, M/F ratio 25/10). We subdivided the study patients into group A (n = 18, mean age 39.6 + 10.6 years, M/F ratio 8/10) and group B patients (n = 28, mean age 45.6 + 8.9 years, M/F ratio 23/5) who had normal and abnormal values of creatinine clearance respectively. An enzyme-linked-immunosorbent assay (ELISA) was used to quantitate plasma beta2M in the study patients and controls. The median 132M levels of the study patients and controls were 44.0 and 1.7 mg/l respectively (p < 0.0001). Beta-2-M levels correlated significantly with serum creatinine (r = 0.56, p < 0.0001), and creatinine clearance (r = -0.6, p < 0.0001). In group A patients, the median beta2M level was significantly higher than normal (4.1 vs. 1.7 mg/1, p < 0.01). Plasma beta2M levels did not correlate well with any other parameter measured in group A patients. When groups A and B were compared, the median plasma beta2M level in group B was significantly higher than group A (20.3 vs. 4.1 mg/1, p < 0.0001). The urinary beta2M (expressed per mg urine creatinine) was also higher in group B than group A patients (6.8 vs. 0.7 p < 0.05). We conclude that elevation of beta2M-microglobulin in patients with the nephrotic syndrome who have normal creatinine clearance suggests early abnormal renal function in these patients. It may be used to assess the rate of normalisation of renal function or progression to chronic renal failure.

Acute hyponatremic encephalopathy after a cerebrovascular accident.

A 66-year-old hypertensive male with acute intracerebral hemorrhage developed acute hyponatremic coma 3 days after the addition of enalapril and a combination of amiloride and a thiazide diuretic to his hypotensive regimen. The patient recovered consciousness and serum sodium normalized 2 days after fluid restriction and withdrawal of both medications. Three weeks later, upon inadvertent reinstitution of enalapril and indapamide, severe hyponatremic encephalopathy promptly recurred; recovery was again rapid following fluid restriction and withdrawal of both medications. This temporal relationship establishes the thiazide diuretic or the angiotensin converting enzyme inhibitor or both as the cause of the profound symptomatic hyponatremia in this patient. Results of simultaneous serum and urine osmolality assays on several occasions were consistent with a decrease in free water clearance, a result of either increased antidiuretic hormone (ADH) secretion or potentiation of its peripheral action, and thiazide-induced natriuresis. The use of a thiazide diuretic in the presence of either of these aberrations of ADH homeostasis most likely explains the profound and rapid development of hyponatremia. Drug-induced disturbances in serum osmolality are a potentially reversible cause of deterioration of the mental state in a patient with an acute cerebrovascular accident.

Xeroderma pigmentosum with adult Wilms' tumour. A rare association.

Xeroderma pigmentosum is an uncommon skin disorder associated with increased incidence of skin tumours and, less often, internal malignancies. Wilms' tumour is a common paediatric tumour, observed rarely in adults. Here we report the rare association of Xeroderma pigmentosum with adult Wilms' tumour in two Libyan females. To the best of our knowledge, this association has not been reported so far in the literature.