ABSTRACT
The authors did not receive any funding for this work.
Subject(s)
Tissue and Organ Procurement , Death , Humans , Tissue DonorsABSTRACT
BACKGROUND: Graft primary non-function (PNF) is the most severe complication after orthotopic liver transplantation (OLT) and is frequently associated with livers from uncontrolled circulatory death (uDCD). METHODS: We reviewed retrospectively the incidence, risk factors, and outcome of patients showing PNF after receiving uDCD liver grafts. The series comprises 75 OLT performed during 11 years. RESULTS: The incidence of PNF using uDCD livers was 8%. We compared patients who developed PNF (n = 6) vs. patients without PNF (n = 69). Mean pump flow of donors during normothermic regional perfusion (NRP) was significantly lower in PNF (p = .032). Day 1 post-OLT levels of transaminases and the incidence of renal complications and postoperative mortality were also significantly higher in the PNF group, but 5-year patient survival was similar in both groups (66.7% in PNF and 68.5% in non-PNF). All PNF patients underwent re-OLT, and 2 died. PNF incidence has decreased in the last 5-years. Binary logistic regression analysis confirmed final ALT value >4 times the normal value as risk factor for PNF, and median donor pump flow >3700 ml/min as protective effect. CONCLUSIONS: Adequate donor pump flow during NRP was a protective.
Subject(s)
Liver Transplantation , Graft Survival , Humans , Incidence , Liver Transplantation/adverse effects , Retrospective Studies , Risk Factors , Tissue DonorsABSTRACT
Cartilage-hair hypoplasia (CHH), or McKusick type metaphyseal chondrodysplasia, was first recognized as a distinct entity in the Old Order Amish in the USA, but was later identified in other groups, and found to be unusually frequent among Finns. CHH is highly pleiotropic with manifestations that include short stature, defective cellular immunity and predisposition to several cancers. CHH is caused by mutations in the RNA component of RNase MRP (RMRP, ribonuclease mitochondrial RNA processing) and is transmitted as an autosomal recessive trait. In the present work, a Spanish CHH patient was extensively characterized at the immunological and molecular DNA level. Several parameters of cellular and humoral immunity were analyzed in this patient: lymphocyte subpopulation, proliferative responsiveness in mitogen stimulation and quantification of serum immunoglobulins. Sequencing of the RMRP gene allowed identification of two mutations in the patient: a +4 C>T substitution previously described on one allele, and a duplication of 15 nucleotides at position -11 on the other allele. This mutation has not previously been described.
Subject(s)
Cartilage/abnormalities , Endoribonucleases/genetics , Hair/abnormalities , Osteochondrodysplasias/genetics , Osteochondrodysplasias/immunology , Base Sequence , DNA Mutational Analysis , Female , Humans , Infant , Molecular Sequence Data , Mutation , Osteochondrodysplasias/physiopathology , SpainABSTRACT
We present a case of a chronic hepatitis C with damage of bile ducts resembling primary biliary cirrhosis. The immunological profile (negative antimitochondrial antibodies and positive anti-nuclear antibody) was characteristic of the autoimmune cholangitis. One year of treatment with ursodeoxycholic acid returned the liver tests to the normal range but the liver lesions remained unchanged.