[Recurrent unexplained lipothymia: do not forget the capillary leak syndrome]. / Lipothymie récidivante inexpliquée: penser au syndrome d'hyperperméabilité capillaire idiopathique.

INTRODUCTION: Systemic capillary leak syndrome (SCLS) is a rare disorder characterized by recurrent spontaneous episodes of hypovolaemic shock due to marked plasma shifts from the intravascular to the extravascular space. It presents as the characteristic triad of hypotension, haemoconcentration and hypoalbuminemia. CASE REPORT: We describe a patient with SCLS with recurrent lipothymia who presented first with delayed oedema that was thought to be due to orlistat treatment. On the second episode the patient was seen with a pulmonary hypertension when plasma came back into vessels. On the third time the characteristic triad led to the diagnosis of SCLS. DISCUSSION: SCLS should be considered in the differential diagnosis of recurrent hypovolemic shock without identifiable cause. Nevertheless, symptoms may be restricted to sole lipothymia or transient oedema or delayed hypoalbuminemia rendering the diagnosis difficult.

Metabolism of phenylalanine in mice homozygous for the gene 'dilute lethal'.

Mice homozygous for d(l) have been suggested as models for phenylketonuria. We found: (1) the concentration of phenylalanine in the blood was normal at all ages examined; (2) phenylalanine hydroxylase activity in the liver in vitro equalled that in unaffected littermates; (3) the apparent K(m) values for phenylalanine and cofactor respectively in d(l)/d(l) mice were the same as in their normal littermates; (4) inhibition of the overall reaction by the particulate fraction, excess of substrate, excess of cofactor or phenylpyruvic acid showed no difference between d(l)/d(l) mice and their unaffected littermates; (5) phenylalanine injected in vivo had equal, small, effects on phenylalanine hydroxylase activity of the liver measured in vitro in the two groups of mice. An explanation of the findings of other workers, based on the natural history of the disease process, is tentatively put forward.