Hepatic involvement is a common feature in childhood mitochondrial hepatopathies, particularly in the neonatal period. Respiratory chain disorders may present as neonatal acute liver failure, hepatic steatohepatitis, cholestasis, or cirrhosis with chronic liver failure of insidious onset. In recent years, specific molecular defects (mutations in nuclear genes such as SCO1, BCS1L, POLG, DGUOK, and MPV17 and the deletion or rearrangement of mitochondrial DNA) have been identified, with the promise of genetic and prenatal diagnosis. The current treatment of mitochondrial hepatopathies is largely ineffective, and the prognosis is generally poor. The role of liver transplantation in patients with liver failure remains poorly defined because of the systemic nature of the disease, which does not respond to transplantation. Prospective, longitudinal, multicentered studies will be needed to address the gaps in our knowledge in these rare liver diseases.
Liver involvement, a common feature in childhood mitochondrial hepatopathies, particularly in the neonatal period, may manifest as neonatal acute liver failure, hepatic steatohepatitis, cholestasis, or cirrhosis with chronic liver failure of insidious onset. There are usually significant neuromuscular symptoms, multisystem involvement, and lactic acidemia. The liver disease is usually progressive and eventually fatal. Current medical therapy of mitochondrial hepatopathies is largely ineffective, and the prognosis is usually poor. The role of liver transplantation in patients with liver failure remains poorly defined because of the systemic nature of the disease that does not respond to transplantation. Several specific molecular defects (mutations in nuclear genes such as SCO1, BCS1L, POLG, DGUOK, and MPV17 and deletion or rearrangement of mitochondrial DNA) have been identified in recent years. Prospective, longitudinal multicenter studies will be needed to address the gaps in our knowledge in these rare liver diseases.
Tri-parent baby technology is an assisted reproductive treatment which aims to minimize or eliminate maternal inheritance of mutated mitochondrial DNA (mtDNA). The technology became popular following the move by the United Kingdom in granting license to a group of researchers from the Newcastle Fertility Centre, Newcastle University to conduct research on the symptoms of defective mtDNA. This technology differs from other assisted reproductive technology because it involves the use of gamete components retrieved from three different individuals. Indirectly, it affects the preservation of lineage which is important from an Islamic point of view. This paper aims to analyze and discuss the implications of the tri-parent technology on preservation of lineage from the perspective of Maqasid al-Shari'ah based the Islamic bioethics. The analysis shows that there are a few violations of the preservation of lineage, hence the tri-parent baby technology should not be permitted.