Abstract
A girl was followed from birth to age 6 y because of congenital heart defect and persisting hypergalactosemia without enzyme defect. An open ductus venosus was suspected, remained undetected by conventional 2-dimensional ultrasonography but was demonstrated by combined color and pulsed wave Doppler sonography at age 4 y. Average forward flow in the portocaval shunt was 240 ml/min; intrahepatic venous flow or other intra- or retroperitoneal venous collaterals were not seen. Episodes of sleepiness, withdrawal, apathy, occasional vomiting, complaints of headache inbetween, starting at age 3.5 y, were first misinterpreted as migraine. After age 4, she occasionally smelled of ammonia, had mild intention and head tremor, dysmetria, rubeosis of face, hands and feet, insecurity of gait, moderate hyperammonemia, elevated liver enzymes in serum. Her liver remained small, by ultrasound. She was treated with low-protein diet, lactulose, sodium benzoate, with limited success. Tentative treatment with flumazenil is in progress. Hypergalactosemia without enzyme defect is an early sign of duct persistence. The early onset of portosystemic encephalopathy correlates with the large volume of shunted splanchnic blood.
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Gitzelmann, R., Arbenz, U., Willi, U. et al. 139 PORTOSYSTEMIC ENCEPHALOPATHY DUE TO PERSISTENCE OF AN OPEN DUCTUS VENOSUS ARANTII. Pediatr Res 30, 651 (1991). https://doi.org/10.1203/00006450-199112000-00169
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DOI: https://doi.org/10.1203/00006450-199112000-00169
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