Nephrotic syndrome in the course of treatment of Wilson's disease with DL-penicillamine.

At the age of 8 years, a girl, who was of Jewish-Yemenite origin, had transient jaundice which was diagnosed as infective hepatitis. During the following 1j years she felt well, except for slight pain in the liver region. At the age of 9j she was admitted for investigation, when a diagnosis of hepatic type of Wilson's disease was made. There was no jaundice. Neurological examination and EEG examination were normal. The liver was palpable 3 cm. below the right costal margin. A Kayser-Fleischer ring was present. Kidney function tests were normal. There was no aminoaciduria. Liver flocculation tests were positive 1 +, SGOT 100-120 units. Retention of bromsulphalein was 46% in 5 minutes and 19% in 45 minutes. Caeruloplasmin in the serum was 0 * 05 oxidase activity optical units (normal for our laboratory, 0 1-0 3). Copper excretion in the urine 249 Mg./24 hr.; serum copper 50-86 ,g./100 ml. The patient's younger sister was first examined in our department at the age of 7; she was found to have a low serum caeruloplasmin level, and high excretion of copper in the urine. There was no Kayser-Fleischer ring and the liver tests were almost normal. She is now undergoing further investigation. The parents and the other 5 children were all normal. After the diagnosis of Wilson's disease had been confirmed, the patient was treated with 0 * 5 g. DLpenicillamine per day. This treatment was given regularly for 3 months, during which time the copper excretion in the urine rose to an average of 700 Mg./24 hours. During the following 10 months, she did not receive continuous treatment as she failed to consult her