ABSTRACT
The main indication for orthotopic liver transplantation (OLTx) in Wilson's disease (WD) is severe hepatic decompensation. Our 15-year-old patient is the second case to date in whom OLTx was performed because of neurologic manifestations resulting from WD. His initial condition involving recurrent headaches, tremor, and athetoid hand movements progressively deteriorated during therapy with D-penicillamine, zinc sulfate, and trientine until he was severely dysarthric, unable to walk, and bedridden. After OLTx, his neurologic condition became almost normal.
Subject(s)
Brain Diseases/therapy , Hepatolenticular Degeneration/therapy , Liver Transplantation , Adolescent , Brain Diseases/complications , Copper/metabolism , Hepatolenticular Degeneration/complications , Hepatolenticular Degeneration/metabolism , Humans , MaleABSTRACT
Oxycardiorespirographies, recording arterial oxygen saturation (SaO2), breathing movements, heart rate and ECG with a mean recording time of 22.3 hours, were performed on 85 preterm (mean postconceptional age: 38 weeks) and 81 term infants (mean postconceptional age 42.4 weeks). 83% of the preterm infants showed periodic breathing (PB), in 97% of them this was accompanied by periodic variations of arterial oxygen saturation (PVO). Periodic breathing occurred in 61% of the term infants, 84% of them showed PVO during periodic breathing. The mean variation of oxygen saturation was between 92.8 and 96.8% (+/- 1.7) for preterm and between 92.9 and 96.0% (+/- 2.2) for term infants. In some infants the peak to peak amplitude of the SaO2 cycles was up to 22%, sometimes a further fall of SaO2 occurred. There was a strong correlation of the PVO both at the beginning and end of the episode as well as with the PB-cycle periodicity itself. The fall of the oxygen saturation occurred 3.1 to 7.8 s after the beginning of the first apnea of an episode of periodic breathing, the minimum SaO2 was reached approximately 4.2 to 8.6 s later. This periodic rapid fall of SaO2 from a high oxygenation level cannot be explained by the apneas of a rather short duration during periodic breathing. It is discussed that PVO during periodic breathing may be caused by an ideopathic right to left shunting across fetal circulation pathways which occurs intermittently and periodically. This mechanism could-via patterns of reaction exhibited during the fetal and neonatal time period-lead to acute hypoxemia, as found in apparently life threatening events (ALTE) and as postulated in sudden infant death (SID).
