Laryngeal papillomas: the epidemiology in a Danish subpopulation 1965–1984

The incidence rate of laryngeal papillomas in a Danish subpopulation (approximately 2.8 million inhabitants) was 3.84 × 10^-6 per year in the period 1968–1984. For juvenile papillomas the incidence rate was 3.62 × 10^-6, compared with 3.94 × 10^-6 for laryngeal papillomas of adult onset. When comparing different time periods a significantly low incidence was found in the time 1965–1968, while the incidence remained constant in 1969–1984. The low incidence rate in the early period may be real, but selectional bias may have played a part. It is in general anticipated that maternal genital HPV-infections may serve as an HPV-reservoir, and that juvenile laryngeal papilloma is a result of HPV transmission from the mother to the child during birth. In the period in question cervical HPV-infections have been recorded with increasing frequency in younger women, indicating that the prevalence is rising. However, this is not reflected in the incidence of laryngeal papillomas.     

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.     

Inhibition of the effects of thyroxine by 5,5-diphenyl-2-thiohydantoin

5,5-Diphenyl-2-thiohydantoin (DPTH), the sulfur analog of 5,5-diphenylhydantoin (dilantin), inhibited the induction of rat tissue mitochondrial α-glycerophosphate dehydrogenase (GPD) and the stimulation of whole-body metabolic rate by exogenous thyroxine (T₄). It did not affect the tissue uptake, the biliary excretion, or the enterohepatic recirculation of T₄. Like propylthiouracil, it increased the serum protein-bound iodine (and free T₄) when exogenous T₄ was given, and decreased the deiodination in vivo of the hormone. In thyroidectomized rats, DPTH did not inhibit the stimulation of general protein synthesis produced by exogenous T₄, and it had only a little or no effect on the induction of glucose 6-phosphatase, succinic dehydrogenase, and malic enzyme by T₄, or on the induction of tyrosine transaminase by hydrocortisone. The inhibition of GPD induction by DPTH could not be explained by its effects on the general distribution or metabolism of T₄.