Patients with Klippel-Trenaunay (KT) syndrome have a complex constellation of anomalies that includes cutaneous capillary malformation (usually on an affected limb), abnormal development of the deep and superficial veins, and limb asymmetry, usually enlargement. Mixed vascular malformations may be present and include capillary, venous, arterial, and lymphatic systems. The records of 79 patients referred for vascular anomalies were reviewed and 49 were found to have the three "cardinal" anomalies of KT syndrome. Twenty-six females and 23 males had 46 affected legs (27 right legs), 23 affected arms (15 right), 21 affected trunks, and 10 affected heads. Thirty-six had only one affected quadrant, 8 had two, and 5 had three or more. Although 40 patients had increased limb girth, measurable length discrepancy was noted in only 17 individuals. Patients were evaluated using a noninvasive imaging strategy including color duplex ultrasonography, MRI, lymphoscintigraphy, and plain radiographs. Treatment included compression, pulsed-dye laser treatment, reduction of arteriovenous malformations, and orthopedic procedures for overgrowth. All KT cases in this series occurred sporadically. We speculate that KT syndromes may be due to a somatic mutation for a factor critical to vasculogenesis and angiogenesis in embryonic development.

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The in vitro metabolizing method was modified and its ability to correctly identify eight known hemolytic and nine known nonhemolytic drugs of glucose-6-phosphate (G6PD)-deficient erythrocytes was evaluated. The technique is based on inducing in vitro drug metabolism by incubation of red cells and drug with a reduced NADP-generating system in the presence of phenobarbital-induced mouse liver microsomes. Thus, this system provides a model for in vivo metabolic function. The hemolytic potential of tested drugs is indicated by the extent of loss of reduced glutathione of G6PD-deficient erythrocytes during 60-min incubations. Complete agreement between the test and literature for nonhemolytic compounds was observed. The test also correctly identified six of the eight known hemolytic drugs and failed to identify two known hemolytic drugs (acetanilide and sulfacetamide). The test was also applied to 14 drugs about which there is uncertainty regarding hemolytic potential. Of the latter, DL-alpha-methyldopa; alpha-naphthol; beta-naphthol; 2,3, dimercaptopropanol; phenacetin; and menadione were found to react positively. We conclude that this in vitro assay system will be useful in predicting which new drugs will be hemolytic in G6PD-deficient patients.

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