Liver transplantation in tyrosinaemia type 1: the dilemma of timing the operation

Four children with tyrosinaemia type 1 received liver transplants. The metabolic disorder was corrected and all four had normal liver function on an unrestricted diet. Two children, transplanted at age five and seven years, proved to have occult hepatocellular carcinoma and both subsequently developed pulmonary metastases. One child was well 32 months after removal of a single pulmonary metastasis but the other child died with multiple metastases. The two younger children, transplanted at age 19 and 21 months, were well 28 and 44 months after operation, one after a second liver transplant. Our experience confirms the high risk of hepatocellular carcinoma in this disease and the potential value of early liver transplantation.     

Intrapulmonary shunting in the biliary atresia/polysplenia syndrome: reversal after liver transplantation

One hundred and seventy three children, including 93 with biliary atresia, received liver grafts at Addenbrooke's Hospital between 1983 and 1993. Of these, only seven developed cyanosis due to intrapulmonary shunting as a complication of their liver disease, and all seven of these had the biliary atresia/polysplenia syndrome. Intrapulmonary shunting was confirmed by a radioisotope scan in four children. Only one child with the syndrome did not have cyanosis when undergoing transplantation. Seven of the eight children are alive 6-54 months after transplantation, with normal pulmonary and hepatic function. Cyanosis recurred in one child who developed chronic rejection with liver failure. In conclusion: (a) there is a strong association between the biliary atresia/polysplenia syndrome and cyanosis due to intrapulmonary shunting; (b) intrapulmonary shunting is fully reversible after successful liver transplantation; and (c) cyanosis, once present, is progressive, and these children should be considered for liver transplantation as soon as it occurs.     

Neurological crisis in hereditary tyrosinaemia and complete reversal after liver transplantation

A 19 month old Indian girl with tyrosinaemia developed a severe generalised neuropathy involving both phrenic nerves. Treatment with haemarginate failed to improve her condition. After liver transplantation the raised concentrations of the neurotoxin delta amino-laevulinic acid returned to normal and gradual but complete neurological recovery occurred over a period of 13 months.     

Early hypothyroidism after subtotal thyroidectomy in patients with Graves' disease--the role of the preoperative conservative treatment and hormonal status

AIM: The aim of the present study was to evaluate the role of the preoperative antithyroid drug treatment and hormonal status in the development of early postoperative hypothyroidism after subtotal thyroidectomy in patients with Graves' disease. MATERIAL AND METHODS: Eighty-five patients with Graves' disease (males : females ratio 1:5.54, age range 19 to 64, 37.52 +/- 1.09 yrs) who had previously undergone surgical treatment were enrolled in the study. All patients underwent bilateral subtotal thyroidectomy with the amount of remnant tissue of 2-3 g for each lobe (total 4-6 g). Development of early (within one year after the operation) postoperative hypothyroidism was analyzed regarding the type of the antithyroid drug, preoperative dose, duration of the preoperative medical treatment, FT3, FT4, FT3/FT4 and hTSH. RESULTS: Forty six percent of all examined patients (54.12%) were euthyroid and 39 (45.88%/)--hypothyroid. Postoperative hypothyroidism was developed by 33.33% of the patients that had received preoperatively propylthiouracil compared with 50.82% of those treated with methymazol (p > 0.05). The duration of the preoperative treatment was 38.36 +/- 3.53 months for the hypothyroid patients and 30.11 +/- 2.34 months for the euthyroid patients (p < 0.05). Postoperative hypothyroidism developed in 58.70% of the patients with preoperatively suppressed thyroid-stimulating hormone (hTSH) and in 33.33% of those with normalized values of hTSH (p < 0.05). No statistically significant between-group difference was found in the preoperative dose of antithyroid agent, mean values of free triiodothyronine (FT3), free thyroxine (FT4), FT3/FT4, thyrotropic hormone (TSH). CONCLUSIONS: Longer preoperative antithyroid drug treatment and suppression of hTSH in the preoperative period correlated with higher risk of hypothyroidism after subtotal thyroidectomy. The type and the preoperative dose of the antithyroid agent, as well as the mean values of thyroid hormones before the operation have no prognostic significance for postoperative thyroid hypofunction.