Pathogenesis of "nephrotic crisis" in children.

Abstract

We examined 20 episodes of nephrotic crisis (Cx) in 13 nephrotic children and report hematological findings and pathogenesis of the crisis. All physical and clinical data including serum and plasma protein levels and urinalysis, were recorded at the onset of Cx, during recovery and the interventing interval. There was a total of 20 incidences of Cx: 17 occurred at relapses and 3 at the onset of the nephrotic syndrome. We compare the Cx data with the data we additionally collected from 14 other children suf f erring with nephrotic syndrome but without the complication of Cx (non-Cx) either at relapses (10) or first onset (4). The most characteristic Cx clinical manifestations (compared with non-Cx group) were abdominal pain in 85 % (cf. 14.3 %), vomiting in 70% (cf. 0 %), and facial pallor in 65% (cf. 7.1 %). There were significant differences of the Cx clinical and hematological data when compared with the non-Cx data. The Cx group showed a significantly lower blood pressure, higher hematocrit value, lower serum sodium concentration, lower C3 level, lower CH50 activity and lower serum total protein and albumin (standardized for hematocrit values). We conclude that hypovolemia was the pathogenesis for Cx: the sudden loss of massive serum protein into urine, causing subsequent hypoproteinemia (hypoalbuminemia) and decreased colloidal osmotic pressure impairing re-entry transport of interstitial fluids from tissue to the blood. The hypovolemia of Cx is aggravated by the hyponatremia which arises due to a sodium shift from the blood to interstitial tissue. We investigated the role and mechanism of a possible hypoadrenocortical state and report negative findings in all cases.