Coagulation studies were performed in 16 children with steroid responsive minimal change nephrotic syndrome in order to elucidate the incidence of thromboembolic complications. Fibrinogen and alpha 2-macroglobulin concentrations were inversely correlated with serum albumin concentrations, antithrombin III correlated positively (p less than 0.001). Factor VIII:R:AG concentration was elevated. Coagulation disturbances in children are not less severe than in adults with nephrotic syndrome. Combined scintigraphic pulmonary ventilation and perfusion studies were employed in 26 children to detect noninvasively events of pulmonary embolism, respectively their residual changes. The lung scintigraphic investigation demonstrated a pattern consistent with pulmonary embolism in 7 patients (27.9%), residual changes in 10 (38.5%) and normal findings in 9 (34.9%). The incidence of thromboembolic complications in children with severe nephrotic syndrome is as high as reported for adults. Pulmonary symptoms may well be due to pulmonary embolism.

The segmental ventilation-perfusion mismatch is almost invariably a sign of pulmonary embolism (PE). As ventilation scintigraphy is an expensive and time-consuming procedure, it is rarely performed in patients presenting as emergencies. In such patients PE is diagnosed by the presence of a segmental perfusion defect (SPD) in combination with a normal chest X-ray. However, little is known about SPD frequencies in different groups of patients or its value in predicting a mismatch. To determine this frequency, as well as its predictive value for a mismatch (PVM), we examined 764 patients with suspected Pe (PE? group), 359 patients at low risk for PE (low risk group), and 234 patients without suspected PE but at high risk for PE (high risk group), all by perfusion and ventilation scintigraphy. Frequencies of SPD were determined in each group as a function of age. PVM was calculated for each age subgroup using Bayes' theorem. In the low risk group the frequency of segmental mismatches (SM) was about 4%, while one-third of patients at high risk had a SM. This was not age dependent in contrast to the PE? group, where the frequency decreased with age. In the youngest subgroups the PVM for a SPD was higher than 0.9 as well as in a subgroup of the PE? group with a known thrombo-embolic disease. In these subgroups a ventilation scintigraphy is not required for the scintigraphic diagnosis of PE; in all other patients an additional ventilation study is necessary.(ABSTRACT TRUNCATED AT 250 WORDS)

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