Autoimmune haemolysis as an unusual cause of anaemia in von Recklinghausen's disease.

Von Recklinghausen's disease, now classified as neurofibromatosis type 1 (NF-1), is a relatively frequent autosomal dominant disorder and has clinical manifestations, such as cafe-au-lait spots, freckling, generalised cutaneus neurofibroma, Lisch nodules, short stature, optic glioma and central nervous system tumours. In adults, anaemia in the course of NF-1 is usually due to gastrointestinal tumour bleeding. Association of NF-1 and autoimmune haemolytic anaemia is unusual. Here, we report a 48-year-old woman with NF-1 presenting as autoimmune haemolytic anaemia. We also reviewed the literature about the association of NF-1 and autoimmune diseases.

The effect of trimetazidine on intrahepatic cholestasis caused by carmustine in rats.

This study investigated the effect of trimetazidine (TMZ), known as an anti-oxidant agent, on intrahepatic cholestasis caused by Carmustine (BCNU) in rats. Rats were assigned into four groups. The first group (Saline) consisted of 12 rats, which were injected with 2 ml/kg of saline intraperitoneally (IP) 48 h before the study. The second group (corn oil group, n=15), which were injected with 2 ml/kg of corn oil IP 48 h before the study. The third group (BCNU group, n=16), which were injected with 2 ml/kg of corn oil+25 mg/kg BCNU IP 48 h before the study. The fourth group (TMZ group, n=12), which were injected with 2.5 mg/kg per day of TMZ IP, administered at the same hour of the day as a single-dose. Twelve hour after the first dose of TMZ, corn oil 2 ml/kg+BCNU 25 mg/kg IP were injected, and the rats were included in the study 48 h after the administration of corn oil+BCNU. Following a pentobarbital anaesthesia, abdomen was opened with incision, a cannula was placed into the channel of choledocus, and the amount of bile was measured per hour. Then intracardiac blood sample was taken, and consequently centrifuged to obtain the plasma. Finally, the rats were killed with cervical dislocation, and their livers were removed and weighted. In addition to histopathological examination of liver, the levels of malon dialdehyde (MDA), oxidised glutation (GSSG), and reduced glutation (GSH) were detected. Also the osmolality of bile and plasma was estimated in mOsm/kg. As a result, the biliary flow was seen to decrease in BCNU group (P<0.005), but to be normal in TMZ group. The serum level of conjugated biluribin was higher in BCNU group compared to other groups (P<0.05 for each). Although the level of total glutation was lower (P<0.005) in TMZ group, GSH/GSSG ratio was normal. These findings suggest that TMZ has a protective effect on intrahepatic cholestasis caused by BCNU.

Omentoplasty versus introflexion for hydatid liver cysts.

Forty-three patients with unilocular hydatid cyst of the liver were divided into three groups. Omentoplasty (n = 15) was performed in group 1, introflexion (n = 18) in group 2 and a combination of both (n = 10) in group 3. Patients with omentoplasty developed fewer complications and had a significantly shorter hospitalization than those with introflexion. The results indicate that omentoplasty is superior both to introflexion and to introflexion plus omentoplasty for the management of hydatid liver cysts.

The postoperative appearance of the liver on ultrasonography following hydatid cyst surgery.

The postoperative ultrasound pattern in 33 patients with previous surgery for unilocular hydatid disease of the liver was investigated. Each patient was submitted to liver scanning with 99mTc pertecnate, as well as to real time ultrasound examination. The patients were divided into three groups according to the type of surgery performed, namely; omentopexy, introflexion and omentoplexy with introflexion. Postoperative liver scans revealed defects similar to those detected during initial diagnosis in 88% of the patients, regardless of the surgical procedure performed. On the contrary, ultrasonography gave separate specific patterns for each surgical procedure.